CHAPTER 8 Brian M. Kreisman, Jennifer L. Smart, and Andrew B. John |) INTRODUCTION Diagnostic audiology is the use of audiologic tests to determine the location of a problem in the auditory sys- tem and, in many cases, further insights about the disor- der, Diagnostic audiology can be likened to crime shows you may watch on television, Each test serves as a clue that points toward a diagnosis of the patient’s hearing disorder. However, if individual tests (or clues) are examined with- ‘out taking other evidence into consideration, a wrong con- clusion might be made, For audiologists to make correct diagnoses, an audiologic test battery is used. A test battery is a series or combination of tests used to assess the audi- tory system. For most of the examples in this chapter, we will limit discussion of diagnostic audiology to tests that are commonly performed in an audiologic clinic, includ- ing puretone air-conduction and bone-conduction testing, speech testing, tympanometry, acoustic reflex thresholds (ARTS, also called middle-ear muscle reflexes [MEMRs]), and otoacoustic emissions (OAEs). These tests are dis- ‘cussed more fully in other chapters in this textbook and we refer you to these chapters for specific test procedures and norms (see Table 8.1); however, itis important to under- stand how to utilize these tests synergistically to arrive at ‘an accurate diagnosis for each patient. Audiomettic tests are used in conjunction with one another to help reinforce ‘1; alternatively, rule out the diagnosis of a particular type ‘Audiology Procedures Discussed in This Chapter Test Chapter Puretone testing [air and bone 3 conduction) Speech testing (quiet) Speech-in-noise testing Tympanometry ‘Acoustic reflex thresholds Otoacoustic emissions ‘of hearing loss or the site of lesion. The test battery is use- ful for determining some, but not all, auditory disorders. Following a brief introduction to the cross-check pri ciples employed by audiologists, this chapter will utilize a cease study format. Finally, we will address the limitations ‘of the test battery and discus situations when referrals for ‘other testing are indicated. A) CROSS-CHECKING TEST RESULTS ‘The major reason that an audiologist uses a diagnostic bat- tery isto be able to check the results of individual tests with ‘each other. The idea that “the results of a single test are ‘ross-checked by an independent test measure” is referred. to as the cross-check principle (Jerger and Hayes, 1976, p. 614). Since the cross-check principle was first proposed, many manuscripts have revisited the concept as new diag- nostic tests have been developed and different test batteries have been proposed to diagnose specific disorders. The goal ‘of comparing the results of two or more tests isto increase the rate of correct identification of disorders (hit rate) and to decrease the rate of diagnosing a disorder when no disor- der exists (false alarm rate) (Turner, 2003), Cross-checks for Puretone Air Conduction If you only obtained puretone air-conduction thresholds then you would not be able to accurately diagnose the type ‘of hearing loss. Air-conduction audiometry is normally ‘cross checked with bone-conduction audiometry or tympa- nometry to rule outa conductive component of the heating loss. Ifa difference greater than 10 dB exists between the air- conduction and bone-conduction thresholds at the same frequency, a conductive component is indicated. Similarly, air-conduction thresholds for an ear may be within normal limits; however, if a tympanogram for that ear falls outside Of the norms for middle-ear pressure and compliance (eg., Jerger Type B or Type C), a conductive component may be present. ARTs can reveal more information about the type of loss based on the pattern of responses obtained, thus serving as an additional cross-check for puretone air conduction. 19120 _SECTIONI « Basic Tests and Procedures Cross-checks for Puretone Audiometry ‘When puretone audiometry (air- and bone-conduction test. ing) suggests a significant air-bone gap, tympanometry and ARTs can be used to reinforce the diagnosis ofthe conductive clement and to contribute to a specific diagnosis. OAEs also can be used as a cross-check of puretone audiometry. OAEs are used to assess the health of the outer hair cells of the cochlea, but their measurement may be affected by disorders in the conductive pathway. An audiologist might use OAEs as a cross-check to aid in potentially ruling out a nonorganic hearing loss, to verify outer hair cell function and the degree of cochlear hearing loss, and to further assist with the diag. nosis of conductive components, auditory neuropathy spec trum disorder (ANSD), and other etrocochlear disorders. In addition, ARTs have been used to cross check puretone audi ‘ometry (Jerger et al, 1974), although other objective tests, such as tone-burst-stimulated auditory brainstem response (ABR), are considered to be better procedures for estimat- ing hearing thresholds. Acoustic reflexes can be used to help identify the presence of hearing loss in young children as well as in adults with language and/or cognitive issues that may reduce the validity and reliability of behavioral measures (Hall, 2010). Acoustic reflexes can also be used to determine site of lesion within the auditory pathway, specifically in dif. fetentiating between cochlear and retrocochlear pathologies. Cross-check for Puretone Average A puretone average (PTA) is usually calculated as the average of the air-conduction thresholds at 500, 1,000, and 2,000 Hz for each ear. Normally, the PTA should agree with the speech recognition threshold (SRT), meaning that the PTA and SRT should be within 10 dB of one another in the same ear. One instance in which the audiometric thresholds may cause the PTA tobe greater than the SRT by 10 dB is when the audio. gram configuration is sharply sloping or sharply rising. In such instances, itis preferable to use a two-frequency PTA by averaging the two lowest (e., best) thresholds at 500, 1,000, and 2,000 Hz. The two-frequency PTA should then be in agreement with the SRT, Another instance in which the PTA and SRT may disagree is if person is malingering or intentionally exaggerating a hearing loss, Outside of these special circumstances, we would expect SRTS and PTAs to be highly correlated (except when language or foreign lan. guage is a major factor). This allows us to use the SRT to validate the PTA (American Speech-Language-Hearing Association, 1988). Considerations for Assessing Speech Understanding ‘One additional step that audiologists may take to address a patient’s complaint of not being able to understand speech in noisy environments is to administer a speech-in-noise test in addition to the word recognition testing in quiet. Although this is technically not a cross-check, the addition of a speech-in-noise test, especially with sentence stimuli, will provide a more realistic test environment to evaluate a common patient complaint. The puretone audiogram does not necessarily correlate with the amount of difficulty a listener will have in noise (Killion and Niquette, 2000). In addition, when word recognition testing is performed in quiet at a single speech presentation level, no guaran- tee exists that the testis measuring the patient’s maximum speech understanding (Wiley et al, 1995), Cross-check Considerations for Pediatric Testing For children, itis imperative that the audiologist utilize the cross-check principle, The behavioral responses obtained via behavioral observation audiometry (BOA) or visual reinforcement audiometry (VRA) are considered to be accu- rate reflections of a child's true thresholds when these tests are conducted carefully (Madell and Flexer, 2008). How- ever, because children often do not respond as consistently ‘or as quickly as adults, itis possible that a child’s behav- ioral responses may still be elevated compared to actual thresholds. As a result, the audiologist may judge the child’s responses as unreliable (Baldwin et al., 2010). Regardless of the judged reliability of such measures, audiologists should use objective tests such as OAEs and tympanometry as cross-checks for pediatric behavioral responses (Baldwin et al, 2010; Littman et al,, 1998; Madell and Flexer, 2008). In addition, OABs and acoustic reflexes have been shown to be good cross-checks for ABR in young children (Berlin et al,, 2010; Stach et al, 1993). The Joint Committee on Infant Hearing Position Statement (JCIH; American Acad~ cemy of Pediatrics, 2007) also recommends that electro- physiological measures be employed as a cross-check for behavioral response audiometry for children younger than 6 months chronological age. The statement further stresses the importance of obtaining behavioral thresholds as soon as possible using the most age-appropriate method “to cross check and augment physiologic findings” (American Acad- emy of Pediatrics, 2007, p. 910). Electrophysiological Tests as Cross-checks Although beyond the scope of this chapter, it should be noted that certain electrophysiological tests can be used to cross check behavioral measures, as well as to cross check each other and to help confirm diagnoses of certain disor- ders (Bachmann and Hall, 1998; Berlin etal, 2010; Gravel, 2002; Hall and Bondurant, 2009; Stapells, 2011). For exam- ple, Berlin et al. (2010) discussed the use of cross-checkingCHAPTER 8 + Diagnostic Audiology 121 ‘Summary of Cross-checks Used in Diagnostic Audiology Test Test Cross-check Air conduction Puretone audiometry Bone conduction Tympanometry Rule out conductive component (air-bone gap) Helps to verify/rule out middle-ear pathology [ai-bone gaps; rule ‘out nonerganic hearing loss Helps to verfy/rule out middle-ear pathology; helps to confirm outer hair cel function; rule out nonorganic hearing loss Helps to determine ste of lesion (e., differentiate cochlear from retrocochlear hearing loss]; helps to determine degree of hear- ing loss and rule out nonorganic hearing loss Speech recognition Verify performance on both measures (SRT should correlate with Otoacoustic emis- sions Acoustic reflexes Puretone audiometry Puretone audiometry Puretone average threshold. PTA) Speech in quiet {WRS} Speech-in-noise Compare speech perception in quiet [normal auciologic testing) to noise (more realistic test that addresses many patient complaints of not understanding in noise) tests BOA. Electrophysiological measures VRA Electrophysialogical measures Better estimate/confirmation of true thresholds Better estimate of true thresholds {if VRA responses unreliable) Note Tests do not need to be administered inthis order. test results to diagnose ANSD:“...the presence of a [cochlear microphonic] or reversing waves at the beginning of the trace does NOT make a diagnosis of ANSD...without the ‘ross-check of middle-ear muscle reflexes (MEM), OAEs, and an ABR latency-intensity function” (p. 32). For further information about these tests, the reader is referred to the chapters that discuss electrophysiological tests in the text. Table 8.2 summarizes many of the cross-check tests that are used in audiology. Order of Tests Administered Although we acknowledge that there is considerable vari- ability in test protocols actoss clinics, we recommend that testing begin with the objective tests unless contraindi- cated, At least two major advantages can be found for test- ing objective measures first. The first advantage is that the audiologist will have a good idea of the type and degree of hearing loss before beginning the subjective tests. The sec- ‘ond advantage is the reduced risk for misdiagnosis of dis- ‘orders such as ANSD and vestibular schwannoma, as well as failure to detect a patient who is malingering. One caveat needs to be discussed with conducting objective tests frst. With rising costs of health care we need to be cautious that, ‘we are doing tests that are necessary. It is possible that, if the patient’s hearing is completely normal (with no listen- ing complaints) or the patient is profoundly deaf (with previous documentation to support the initial diagnosis), tests such as OAEs and acoustic reflexes will be unlikely to add further information about the patient (but we would recommend that everyone have tympanometry for the reasons previously discussed). We think that a nonorganic ‘component is more likely to be present during subjective testing and may not be discovered until cross-checked with objective tests. For these reasons, we recommend objective testing frst. A suggested testing order is shown in Figure 8.1. Nevertheless, it should be noted that some audiologists advocate giving puretone and speech tests first when the patient may be more alert and can actively respond to these tests and then relax during the objective tests Beyond the Test Battery Although the use of a test battery is important, itis also vital for the audiologist to remember the case history and the patient complaints. In some ways, one may consider this patient-reported information to be a cross-check of the test, battery itself. The case studies presented below demonstrate ‘examples of diagnostic audiology in action. §\\ CASE STUDIES The importance of objective testing in conjunction with subjective tests can be seen through the use of case exam- ples. The following cases are a range of examples that high- light the use and benefit of incorporating the cross-check principle into your clinical practice. The cases will be pre- sented with a brief history and test results, A discussion, ‘of potential difficulties and challenges in interpreting the audiologic data is incorporated within each case. Although122 _ SECTIONI - Basic Tests and Procedures Objective tests 1 Subjective ee FIGURE 8.1 A suggested comprehensive diagnostic test battery there is an ideal order to the test sequence as noted above, the order of tests often varies because of tester preference, patient complaint, age of patient, and so on. Therefore, the test order in the cases below varies to better represent what ‘may be actually done in a clinical setting or what a student clinician may see from his/her supervisors. For the sake of plcity, all of the cases represent testing with standard audiologic procedures on adults, As you read through each ‘case, we encourage you to make a decision tree based on the ‘order of tests presented in the case and then think about how you might decide to change the test order. It is impor- tant to review your clinical decision making periodically to censure that your practice is evidence based. Case1 CASE HISTORY ‘Mr: Ang Kim, age 36, is being seen today after he failed the hearing screening at his company’s health fair. His medical history is generally unremarkable, though he reports that he is just getting over a sinus infection and recently under- ‘went surgery for a slipped disc in his back. You have back- to-back patients today and because there is nothing remark- able in his history you decide to do a quick audiogram and send him on his way. Results from otoscopy, puretone, and speech audiometry are shown in Table 8.3 and Figure 8.2. With subjective information alone this audiogram could indicate many things. For example, you may inac- curately diagnose Mr. Kim with a collapsed ear canal, an impacted cerumen plug, or a perforated tympanic men brane without additional tests to cross check your findings. Otoscopy Findings for Case 1 Right Ear Left Ear Stenotic ear canal, could not visualize tympanic membrane Stenotic ear canal, could not visualize tympanic membrane 20) 40 oo 8 hearing level 20) 400 120 250 500 7,000,000 Frequency (H2) ae pa OT FIGURE 8.2 Puretone and speech audiometry results for case 1 000,000CHAPTER 8 + Diagnostic Audiology 123, Tympanometry Results (226-Hz Probe Tone) for Case 1 ight Left ECV 0.8 mL O7 mL Compliance NP 0.6 mL. Middle-ear pressure NP 50 daPa Transient-evoked Otoacoustic Emission Results for Case 1 1000 1,400 2,000 2,800 4,000 Ear Hz Hz Hz Hz Hz Right Absent Absent Absent Absent Absent Left 10508 10348 N4dB 14908 13.948 Acoustic Reflexes Results (in dB HL] for Case 1 Ipsilateral Contralateral Stimulus Ear 500 Hz 1000Hz ——-2,000Hz S00 Hz 1,000Hz 2,000 Hz Right NR NR NR 110 dB 110.68 105 dB Left 85 dB 80.48 8508 110 dB 105 dB 105 dB NR, no response to maximum presentation (10 d8 HL} Despite your busy schedule, you decide you need more information to make an accurate diagnosis, o you perform, ‘objective testing to cross check your subjective results. The results from immittance testing and OAE testing are shown, in Tables 84-86. With this information, you have several different tests to confirm your finding of a conductive hearing loss. The Type B tympanogram in the right ear reveals normal ear ‘canal volume but no mobility. The normal ear canal volume suggests that the TM is not perforated and there is no ceru- ‘men plug. The pattern of the ARTs is consistent with a right, ‘conductive pathology. TEOAEs in the right ear are absent which is expected with a conductive pathology. The combination of the subjective and objective test results correctly leads you to suspect otitis media with effu- sion and would require a referral for Mr. Kim to a physician, In this case, you ate able to make an appropriate referral based on the information you obtained from a test battery incorporating both objective and subjective measures. Case 2 CASE HISTORY Mrs, Edith Jones, age 77, is being seen today for a hearing test. She does not perceive a listening difficulty but her hus- ‘band was recently ft with hearing aids and insisted she have her hearing checked too. Her medical history is remarkable for high blood pressure and type 2 diabetes which are both, controlled by medication. You conduct a basic audiometric evaluation on Mrs, Jones. Results for otoscopy are displayed in Table 8.7, and puretone and speech audiometry results are shown in Figure 83. Ifyou decide not to proceed with further tests to cross ‘check your results, you might diagnose this patient with normal hearing in the right ear and a mild conductive hear~ ing loss in the left ear. You might then refer Mrs. Jones to an Ear Nose and Throat physician who would order more tests. Instead, you decide to proceed and include additional tests in your battery that would provide a cross-check. We will review those results next (see Tables 8.88.10). These results suggest that Mrs. Jones has normal hear- ing that contradicts your puretone findings. Normal results ‘on tympanometry, ARTs, and TEOAEs are not consistent ‘with a mild conductive hearing loss. With this information you review the patient’ case history and puretone findings again and realize that the apparent conductive hearing loss in the right ea is likely the result of a collapsing ear canal. It is not uncommon for the pressure of the supra-aural head- phones to cause the canal to collapse, particularly in older patients for whom the cartilage supporting the ear canal is Soft. To confirm this finding you decide to retest Mrs. Jones ‘with insert earphones. When you repeat your audiogram using the insert earphones, you measure Mrs. Jones’ right- ‘ear air-conduction thresholds at 5 or 10 dB HL. for all fre- ‘quencies tested. You are able to report to Mrs. Jones that her hearing appears to be normal! Both cases 1 and 2 highlight the importance of using ‘objective test results in conjunction with subjective test results to avoid misdiagnosis. Both audiograms revealed similar test results but very different actual diagnoses, which ‘were only confirmed with the use of objective testing.124 SECTION | + Basic Tests and Procedures 2) FIX eae a aa A 0| 100) FIGURE 8.3. Puretone and speech audiometry results for case 2, 120) 250800 7,000 2000 «4,000 «6,000, Frequency (H2) Otoscopy Findings for Case 2 Tympanometry Results (226-Hz Probe Right Ear Left Ear Tone) for Case 2 Stenotic ear canal, could Stenotic ear canal, could Right Left not tympanic mem- not tympanic mem- ECV 13mL14mb brane brane Compliance O7mL = 08 mL TTTTT_—————~_Hidcte-ear pressure OdaPa daPa Acoustic Reflexes Results (in dB HL) for Case 2 Ipsilateral Contralateral Stimulus Ear 500 Hz 000Hz ——2,000Hz _—_—_—S00Hz 4000Hz 2,000 Hz Right 8548 85 dB 80 dB 90 dB 95 dB 95 dB Left 8508 80.48 25 d8 95 dB 90 dB 90 48 Transient-evoked Otoacoustic Emission Results for Case 2 Ear 1000Hz _1400Hz —_2,000Hz_—_2,800Hz —_ 4,000 Hz Right 8.9.48 91dB123d8 = 10.4dB 7.3.48 Left 9.9.68 10448 10508 9768 6108CHAPTER 8 + Diagnostic Audiology 125 = Otoscopy Findings for Case 3 Clear ear canal, intact tympanic membrane Clear ear canal; intact tympanic membrane Case 3 You receive the following case, accompanied by a patient-signed medical information release, via fax. A recently graduated audiologist at a practice across town, just finished testing Mr. Smith and would like a second opinion. Case HISTORY Mr. Aaron Smith, 49, ports that he can no longer hear out, of his left ear. He works in construction and reports that a transformer overloaded at his work site yesterday, result- ing in a loud explosion which he believes caused his hear- ing loss, Mr. Smith reported that his hearing was normal prior to the explosion. He denies any aural fullness, tinnitus, ‘or dizziness. His medical history is unremarkable and he ‘denies any other injuries asa result of the explosion. Results of the audiologic testing are shown in Tables 8.11-8.13 and Figure 8.4. You call the audiologist right away and review your ‘concerns with her. Both the air-conduction and bone- ‘conduction thresholds for the left ear need to be masked. Cross-hearing should have occurred before those thresh- ‘olds were obtained. Furthermore, you would not expect to obtain no response for bone-conduction testing with the bone oscillator on the left side when the hearing in the right ear is evidently normal. You also note that the PTA, and the SRT are not in agreement for the left ear. ARTS are better than you would expect them to be (given the pur- tone thresholds for the left ear). A patient with hearing thresholds at 90 dB HL would be expected to have ARTS in the range of 95 to 125 dB HL at 500 Hz and 100 to 125, 4B HL at 1,000 and 2,000 Hz (Gelfand et al, 1990). Lastly, Tympanometry Results (226-Hz Probe Tone] for Case 3 ight Left ECV 1.0 mL mL Compliance OS mL 0.6 mL. Middle-ear pressure 5 daPa =20 daPa the WRS was only obtained at 20 dB SL in the left ear, yet Mr. Smith's WRS is 76%, which is better than expected. ‘According to Dubno et al. (1995), a patient with a PTA, ‘of 90 dB HL would have an expected WRS of less than 24%. You suggest to the other audiologist that obtaining TEOAEs would further assist in this diagnosis, The audi- ‘logist performs TEOAES (see Table 8.14) to confirm the suspected diagnosis and faxes the results to you. Based on the pattern of test results, your suspected diagnosis is nonorganic hearing loss. Let us review the facts. First, the patients left ear thresholds are elevated above where cross-hearing should have occurred. Sec- ‘ond, the objective test results (tympanometry, ART, and AEs) reveal no conductive component and suggest that outer hair cells are functioning normally. However, the puretone and speech audiometry results suggest a severe- to-profound unilateral hearing loss in the left eas, which ent with the objective results. Several cros checks identified inconsistencies (e., ARTS and puretones; PIA-SRT agreement; puretone thresholds and OAES). At this point, you could suggest thatthe audiologist reinstruct the patient and then retest the left ear, masking appropri- ately If the thresholds for the let ear ate still elevated, a Stenger test could be performed to confirm the accuracy of the left puretone thresholds. Ifthe Stenger test result is positive (i. the patient does not respond tothe stimulus), this would be additional evidence that the apparent hear- ing loss is nonorganic. This case highlights the importance of a high-quality diagnostic battery (including masking ‘where appropriate) and use of cross-checks to confirm our test results Acoustic Reflexes Results {in dB HL) for Case 3 Ipsilateral Contralateral Stimulus Ear 500 Hz 4000Hz ——_2,000Hz 500 Hz 1,000Hz 2,000 Hz Right 80 a8 85 dB 85 dB 85 dB 90 a8 90 a8 Left 85 dB 80 dB 85 dB 90 a8 85 dB 90 48126 SECTIONI « Basic Tests and Procedures 20) 40) 60] dB hearing level 0] 100 120) 250-600 —«1,000~—«2000 Frequency (H2) one 000 @,000 FIGURE 8.4 Puretone and speech audiometry results for case 3. Transient-evoked Otoacoustic Emission Results for Case 3 Ear 1000Hz _*1,400Hz —2,000Hz_—_—-2,800 Hz 00 Hz Right 9.148 127 48 9.208 10148 12.4 dB Left 10.508 10.348 11.448 149.48 139.48 Case 4 CASE HisToRY MSs. Ashley Jackson, age 27, has had hearing problems all of her life. She has been told by her audiologist that she has only a mild sensory/neural hearing loss. Her doctor always tells her that her hearing is really very good. She tried hear ing aids a few years ago but she says that they did not help atall. Unfortunately, Ms. Jackson cannot hold a job because of her hearing difficulties. Her bosses always cite miscom- ‘munication problems as the reason for her dismissal ‘Ms. Jackson is here today to see if her hearing has changed. Tables 8.15 and 8.16 show otoscopy and tympanometry results, Figure 8.5 shows puretone and speech audiometry results ‘MSs, Jackson's puretone results appear to be consistent with the previous hearing tests in her medical record. There are some red flags that warrant additional testing, though. First, her reports of listening difficulties and communica tion problems in her case history suggest that she may have ‘more than a mild sensory/neural hearing loss. Additionally, her word recognition scores are poorer than expected given her puretone thresholds. You would expect a patient with PTAs in this range to have WRS of 68% or better (Dubno et al, 1995). The next tests that should be performed are ARTs and OAEs. Tables 8.17 and 8.18 show the results of those tests Now that you have completed your testing, you cross check your test results. Fist, the patient’s ARTS are not con- sistent with her puretone thresholds. With a mild sensory/ neural hearing loss you would expect acoustic reflexes to be present (Gelfand et al., 1990). The patient’s TEOAES are present and robust which would not be expected based on ‘Ms. Jackson's puretone thresholds. These findings in con- junction with the poor WRS indicate a need for additional testing such as an ABR. You suspect that the patient has ANSD. Results of the ABR and a medical evaluation may help to confirm or rule out your suspected diagnosis. With- out the addition of both ARTs and OAEs to the test battery, ‘Ms. Jackson's disorder might have been missed again. The recommendations for patients with ANSD or other retroco- chlear pathologies are often very different from the recom- ‘mendations for those who have a peripheral hearing loss. Misidentification of the site of lesion for a hearing loss likeCHAPTER + Diagnostic Audiology 127 Otoscopy Findings for Case 4 Tympanometry Results (226-Hz Probe Right Ear Lett Ear irenel focaeer Clear ear canal; intact Clear ear canal intact uate tympanicmembrane tympanic membrane ECV 14m Compliance 05 mL Middle-ear pressure =10 daPa 2) Fi Ta Tam Boo 100) FIGURE 8.5 Puretone and 250° $001,000 200040008000 speech audiometry results for Frequency (H2) case 4, 120) Acoustic Reflexes Results {in dB HL) for Case 4 Ipsilateral Contralateral Stimulus Ear 500 Hz 4000Hz ——_2,000Hz S00 Hz 1,000Hz 2,000 Hz Right NR NR NR NR NR NR Left NR NR NR NR NR NR NR, no response to maximum presentation (10.48 HU) a ee Transient-evoked Otoacoustic Emission Results for Case 4 Right 20.148 2908 19508 18448 19348 Left 22.508 20648 20.148 229dB 20308128 SECTIONI - Basic Tests and Procedures 20| 40] 60| 4B hearing level 0| 100) 120) 250 500,000,000 Frequency (H2) ‘MBs. Jackson's might result in delayed or inappropriate reha- bilitation recommendations. Unfortunately, ANSD can be ‘missed easly in clinical practice if the audiologist does not perform a thorough test battery (Smart and Kelly, 2008), Case 5 CASE HisToRY ‘Mr, Don Warner, age 58, is being seen today with his pri ‘mary complaint being a constant ringing in his right eat. He notes that the ringing has been present off and on for over 3 years but it has become more bothersome recently. When asked about his hearing he admits that he has difficulty understanding what others are saying in noisy places. He denies aural fullness and dizziness. He plays tennis regularly and generally is in great health. Results from your testing are shown in Tables 8.19-8.22 and Figure 8.6. The order of test administration is important. Because you performed immittance testing and TEOAEs first, you knew that the patient’s tympanic membrane is mobile, that the ART pattern is abnormal in the right ear, and that the ‘outer hair cells of the right ear do not appear to be function- ing normally. You were able to obtain this information before the patient provided any information through subjective test. aT] Otoscopy Findings for Case 5 right Ear Letter Clear ear canal; intact Clear ear canal; intact. tympanic membrane tympanic membrane FIGURE 8.6 Puretone and speech audiometry results for case 5. 0006000 ing. The patient’ asymmetry in high-frequency audiometric thresholds and poor WRS in the right ear combined with the objective results suggest a retrocochlear pathology and ‘warrant an ENT referral for additional testing. The patient's report of unilateral tinnitus, the abnormal ART pattern, the asymmetry in puretone thresholds, and the apparent rollover in the patient’ right-ear word recognition are all suggestive of retrocochlear dysfunction, Taken in isolation, each might be sufficient for you to make a referral for a medical evalu- ation. However, having consistent results from several tests allows you to be more confident in your recommendation and provide the ENT with as much information as possible. Case 6 CASE HISTORY Mr. José Gonzalez, age 24, was seen today for an audiologic evaluation, He was just hired asa clerk fr a federal judge and therefore has to undergo a rigorous physical examination, including hearing test. Mr. Gonzalez denies any hearing dif- ficulties, tinnitus, dizziness, or aural fullness. He reports that he isin great health and is currently training for a marathon. ‘Tympanometry Results (226-Hz Probe Tone) for Case 5 Right Left ECy 1.6 mL 18mL Compliance O7 mL 0.9 mL. Middle-ear pressure OdaPa =10 daPaCHAPTER 8 + Diagnostic Audiology 129 Acoustic Reflexes Results {in dB HL) for Case 5 Ipsilateral Contralateral Stimulus Ear 500 Hz 4000Hz ——-2,000Hz S00 Hz 14000Hz 2,000 Hz Right 105 dB 110 dB 110 dB 110 48 NR NR Left 85 dB 90 a8 85 dB 95 dB 95 dB 95 dB NR, no response to maximum presentation (10 48 HL} Transient-evoked Otoacoustic Emission results for Case 5 Ear 4O00Hz —1400Hz —_2,000Hz —_-2,800Hz 4,000 Hz Right Absent Absent — Absent. ~—Absent_ — Absent Left 8.008 9.3.68 9468 6208 6108 2 == a po _ 400 120 250600 F000 2,000 Frequency (H2) 4.000) FIGURE 8.7 Puretone and speech audiometry results for case 6. 3.000 Tympanometry Results (226-Hz Probe Tone] for Case 6 Otoscopy Findings for Case 6 Right Left Right Ear Left Ear ECV 15 mb 17mb Clear ear canal;intact Clear ear canal intact Compliance 09 mL 7 mL tympanicmembrane tympanic membrane Middle-ear pressure 10 daPa___——15 daPa130 SECTIONI - Basic Tests and Procedures Acoustic Reflexes Results {in dB HL) for Case 6 Ipsilateral Contralateral Stimulus Ear 500 Hz 4000Hz ——2,000Hz ‘S00 Hz 1000Hz 2,000 Hz Right. 85 dB 8548 90 dB 95 dB 90 a8 9548 Left 85 dB 90.48 285 68 90 dB 95 dB 9548 Transient-evoked Otoacoustic Emission Results for Case 6 Ear 4000Hz _*1,400Hz _—-2,000Hz 2,800 Hz 100 Hz Right 123 dB, 14.6 68 10248 11148 8.948 Left 13.508 12848 124 dB 10.1 48 9.9.68 Based on the testing you have completed thus far you ‘would expect this patient has normal hearing. His case his tory and all objective tests suggest hearing within normal limits. You proceed with your puretone and speech testing. Results from your testing are shown in Tables 8.23-8.26 and Figure 8.7. The puretone and speech audiometry results are sur: prising because they contlict with the results from the objec tive tests, Specifically, ARTs and TEOAEs within normal lim: its are not consistent with a measured profound hearing loss in the left ear. Your first thought is nonorganic hearing loss, You decide to reinstruct Me. Gonzalez and retest the left ear air-conduction thresholds. This time you tell Mr. Gonzalez that you are going to play some more beeps in his left ear and allhe has to do is press the button when he hears the tone. He nods and appears to understand the instructions. You begin retesting at 1,000 Hz and Mr. Gonzalez does not respond at the maximum limits of the audiometer. As you enter the booth to reinstruct again, Mr. Gonzalez informs you that he never heard a beep and has been waiting for you to present the tone. In fact, he notes that he has not heard anything from the left earphone. You check the headphone jack con: nections and find that the let headphone jack is unplugged. After you plug in the jack and retest Mr. Gonzalez’s left ear, you obtain thresholds within normal limits, It is important to note that the patient history and the objective test results ‘were not consistent with the subjective test results. Although hhaving a well-constructed test battery is important, you also ‘want to be flexible with your test order and be vigilant to notice inconsistencies between test results as you go. This flexibility would allow you to notice the unplugged head: phone jack sooner and save time and frustration for you and ‘Mr. Gonzalez, A) LIMITATIONS OF THE AUDIOLOGIC TEST BATTERY The combination of well-validated test measures, precise patient instruction, careful scoring, and application of the cross-check principle should result in accurate diagnostic and rehabilitative decisions for most patients, It is impor- tant to remember, however, that real-world patients usually. do not present as textbook cases. The case studies contained in this chapter and the diagnostic criteria published in the audiologic test literature should be treated as guidelines rather than absolute rules. High-quality diagnosis depends ‘on both the construction of a high-quality test battery and skill in interpreting ambiguous or seemingly contradictory test results. A good rule for daily practice is this: When test results seem in disagreement, first check the tester (rule out the clinician's own mistakes); then, check the equipment (rule out malfunction or equipment performing out of calibration); and finaly, check the patient (rule out patient error or pseudohypacusis) §\) MAKING REFERRALS ‘No audiologist is an island. A team approach to the treat- ‘ment of hearing and balance disorders, particularly in pe atric patients, is often indicated. Appropriate treatment of a patient seen for audiologic evaluation may require consultation with specialists including (but not limited to) allergists, endocrinologists, neurologists, occupational therapists, ophthalmologists, psychiatrists, rheumatologists, and speech-language pathologists. Referral of pediatric patients with hearing loss to an ophthalmologist is particu- larly important; approximately 50% of children born withCHAPTER 8 + Diagnostic Audiology 131 severe-to-profound hearing loss also have abnormalities of vision (American Academy of Pediatrics, 2007) Referral for Medical Otolaryngologic Evaluation The most common referral made by audiologists is to a medical doctor. Sending a patient to an otolaryngologist, primary care physician, or pediatrician is indicated if the audiologic evaluation reveals evidence of an underlying medical condition, Symptoms may include ear pain, bleed- ing oF drainage from the ear (otorthea), tympanometeic abnormality without known etiology, or physical abnormal- ity observed during otoscopy: Patients who report frequent ‘ar infections, luctuating or sudden hearing loss, or bal- ance disturbance should also be seen by a medical profes- sional (see Table 8.27). Newly identified hearing loss is also reason for referral. Although some audiologists undertake ‘erumen management in their own practice, many others prefer to refer to an otolaryngologist oF the patients pri- mary care physician for removal of impacted wax. Children ‘who exhibit a previously undiagnosed hearing loss or who ‘exhibit delays in speech or language development should be seen by a pediatric otolaryngologist or developmental pedi atrican prior to any audiologic management. With respect to the audiologic test battery, disagree ‘ment among objective and subjective test measures which ‘cannot be resolved as tester, equipment, or patient error is indicative of need for medical referral. Abnormally poor speech scores relative tothe audiogram, asymmetric hearing loss, and reports of aural fullness and/or tinnitus are other signs of possible serious ear disease which should be evalu- ated bya physician, Referral for Auditory Processing Evaluation Disagreement between objective and subjective hearing tests may be reason to refer a patient for an evaluation of audi- tory processing, Patients with apparently normal peripheral auditory function may still have difficulty processing com- plex signals such as speech. These individuals often report that they can hear wel, but have difficulty understanding What others are saying, particularly inthe presence of noise. Tests of speech perception in noise such as the Bamford~ Kowal-Bench Speech-in-Noise Test (BKB-SIN; Etymotic Research, 2005), Quick Speech-in-Noise Test (QuickSIN; Etymotic Research, 2001), and Hearing in Noise Test (HINT; Nilsson etal, 1994) may help to contirm this difficulty. It performance on speech-in-noise tests is poor, particularly if Seven Signs of Serious Ear Disease Sign Possible Etiologies Ear pain [otalgia) or sensation of fullness Otalgia may be a result of disease of the ear (eg, otitis media, otitis externa) or may be referred pain resulting from illness in the head or neck (e.g, temporomangibular joint dysfunction, tumors of the upper digestive tract) Otorrhea and bleeding may result from chronic otitis media, otitis externa, cholestea- toma, and other disorders of the temporal bone. Bleeding from the ear may also be a sign of traumatic injury to the ear or temporal bone tumor Sudden sensory/neural hearing loss may result from vital infection, ischemic event, trauma, or Vill nerve pathology. Progressive hearing loss is associated with immune disorders and viral or bacterial infections. Fluctuating hearing loss is commonly noted in patients with Méniére’s disease ‘Asymmetric hearing loss and/or unilateral tinnitus may be a result of a tumor on the Vill nerve Discharge (otorthea) or bleeding from the ear Sudden or progressive sensory/neural hear~ ing loss, even with recovery ‘Asymmetric hearing between the ears or tinnitus Hearing loss follow- ing injury, exposure to loud sound, or air travel Blunt or penetrating trauma to the head and barotrauma may result in hearing loss that is conductive (disruption of tympanic membrane and/or ossicular chain) or sensory/ neural (disruption of cochlear membranes). Noise-induced sensory/neural hearing loss may be seen after isolated intense sound events (explosions, gunfire] or repeated exposure to loud noise Delayed speech and language development in children is often a result of inability to hear certain sounds of speech. This may result from conductive hearing loss (usualy Slow or abnormal speech development in children related to otitis media) or permanent sensory/neural loss Balance disturbance or Balance disturbance may be a result of otologic [e.g., Méniére’s disease, perilymph dizziness fistula] or neurologic disease (eg. stroke, demyelinating disease) ‘Adapted from Hall |W Il, Muelle HG. (1997) Audologsts'Desk Reference. VoL San Diego, CA: Singular.132 _SECTIONI - Basic Tests and Procedures the audiogram is normal or suggests good hearing sensitiv ity, auditory testing should be performed. Parental concerns about a child’s ability to process speech in noisy or reverber ant places may also indicate need for APD evaluation. Audi tory processing evaluation and rehabilitation are described in Chapters 27 to 30 of this textbook. Referral for Vestibular Evaluation Formal vestibular evaluation may be indicated by patient history oF results of a doctor’s physical evaluation. The symptoms of vestibular dysfunction are often obvious to the patient, but he or she may not realize that they are a relevant part ofthe audiologic case history. Therefore, its important for any audiologist’s case history form to include questions specifically asking about vertigo orbalance problems to elicit this information. Reports of dizziness (particularly recent dizziness), vertigo, or feelings of spinning suggest need for evaluation by a vestibular specialist and/or otolaryngologist. Reports of imbalance (as opposed to vertigo) are also rea son for medical evaluation, but may require treatment by a physical therapist rather than an audiologist. Other specific indicators for vestibular testing include history of exposure to ototoxins (particularly vestibulotoxins, such as aminogly. coside antibiotics), bacterial meningitis, or perilymph fis tula, Patients with genetic conditions such as Pendred syn. drome, Usher syndrome, and CHARGE syndrome are also candidates for vestibular referral, Pediatric patients present ing with inner ear dysplasia of unknown origin or delays in ‘motor or balance skills should also be referred. Vestibular evaluation (Chapter 21) and rehabilitation (Chapter 22) are discussed in detail later in this textbook. Referral for Genetic Evaluation Roughly 0.196 to 0.396 of children are born with some hear ing loss, and about half of these cases appear to be related to some genetic cause. Of these genetic cases, about 30% are syndromic, meaning that they can be related to sets of, clinically recognizable features or symptoms known to co: ‘occur. The remaining 70% of genetic hearing loss cases are characterized by hearing loss in isolation and ate referred to as nonsyndromic. Approximately 50% of cases of autoso- ‘al recessive nonsyndromic hearing loss are due to muta. tion in gap junction beta-2 (GJB2), the gene that encodes the gap junction protein connexin 26 (CX26) (Kelsell etal, 1997). Hearing loss resulting from connexin 26 mutation is typically present from birth and can range in severity from ‘moderate to profound. More than 90 mutations of GJB2 have been identified. Testing for GJB2 mutation is an emergent field in early hearing loss identification, Quick and low-cost screening ‘methods have been developed and are available through ‘many medical centers and genetic testing service providers. It should be noted that many patients and parents may be hesitant to undergo formal genetic testing because of fears that their health insurance costs may increase if a genetic predisposition to disease is found. For these patients, a con: sultation with a genetic counselor may be preferable to a referral to a medical geneticist. |) summary A well-constructed and consistently administered test bat- tery provides the foundation for high-quality audiologic diagnosis and cate. The case studies contained within this chapter are intended to underscore the importance of the diagnostic battery in terms of test selection, test order, and use of cross-checks. When test discrepancies cannot be resolved or a patient presents with complaints or symptoms ‘outside of the audiologist’s scope of practice, a referral to an appropriate specialist is indicated. Consultation with other specialists can also help the audiologist form a more com- plete picture of a patient’s hearing health, increasing the like- lihood of success in audiologic or vestibular rehabilitation, FOOD FOR THOUGHT 1. For each of the cases presented in this chapter, what are some ways that the test order may have affected your thought process regarding the potential diagnoses? 2. How might you modify the order that you administer tests in your test battery, or change particular tests, based ‘on individual patient factors such as age or cognitive ability? 3, Imagine that you notice colleague in your clinic admin- istering tests in an order that you think is unhelpful or ‘omitting tests that would help to differentiate between possible diagnoses. What might you say or do to suggest a different test battery or test sequence? What evidence ‘might you use to support your suggestion? REFERENCES. ‘American Academy of Pediatrics, Joint Committee on Infant Hearing, (2007) Year 2007 position statement: Principles and {guidelines for early hearing detection and intervention pro- ‘grams, Pediatrics, 120, 898-921, ‘American Speech-Language Hearing, Association. 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