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. Author manuscript; available in PMC: 2018 Aug 1.
Published in final edited form as: Am J Med Genet A. 2017 Jun 1;173(8):2210–2218. doi: 10.1002/ajmg.a.38316

Table IV. Auditory test findings and interpretation.

Test Finding Number of ears (percentage) Interpretation
Tympanometry – Middle ear function
Normal 55/74 (74.3%) Normal middle ear function
Negative Pressure 9/74 (12.2%) Eustachian tube dysfunction
Flat – Normal volume 1/74 (1.4%) Stiff middle ear system
Flat – Large volume 9/74 (12.2%) Patent ventilation tube
DPOAE – Cochlear OHC function
Present: broad range 12/64 (18.8%) Intact OHC function
Present: limited range 3/64 (4.7%) Partial OHC function
Absent* 46/64 (71.8%) OHC dysfunction
Noisy 3/64 (4.7%) Uninterpretable
ABR – Auditory nerve and auditory brainstem function
Normal latencies 48/60 (80%) Normal auditory nerve/auditory brainstem function
Borderline abnormal** 5/60 (8.3%) Attributable to degree of HL
Delayed interpeak latencies 6/60 (10%) Retrocochlear
Absent 1/60 (1.6%) Attributable to degree of HL

Data are presented as number of ears (%);

*

an additional eight ears with absent DPOAEs and abnormal tympanometry were not included in this count as indicative of OHC dysfunction;

**

borderline abnormalities included absent waves I or III (n=4 ears) and delayed wave III with all other findings normal (n=1); DPOAE, distortion product otoacoustic emission; OHC, outer hair cells; ABR, auditory brainstem response; HL, hearing loss