Identifying three otopathologies in humans

Hearing-in-noise (HIN) is a primary complaint of both the hearing impaired and the hearing aid user. Both auditory nerve (AN) function and outer hair cell (OHC) function are thought to contribute to HIN, but their relative contributions are still being elucidated. OHCs play a critical role in HIN by fine tuning the motion of the basilar membrane. Further, animal studies suggest that cochlear (auditory) synaptopathy, which is the loss of synaptic contact between hair cells and the AN, may be another cause of HIN difficulty. While there is evidence that cochlear synaptopathy occurs in animal models, there is debate as to whether cochlear synaptopathy is clinically significant in humans, which may be due to disparate methods of measuring noise exposure in humans and our high variability in susceptibility to noise damage. Rather than use self-reported noise exposure to define synaptopathic groups, this paper assumes that the general population exhibits a range of noise exposures and resulting otopathologies and defines cochlear synaptopathy "operationally" as low CAP amplitude accompanied by normal DPOAE levels in persons with low pure tone averages. The first question is whether the standard audiogram detects AN dysfunction and OHC dysfunction? The second question is whether HIN performance is primarily dependent on AN function, OHC function, or both functions?

Adult subjects have been recruited to participate in an ongoing study and variables such as age, self-reported gender, pure tone audiometry (0.25-20 kHz), subjective perception of HIN difficulty, Quick Speech-in Noise (QuickSIN) test, 45% time compressed word recognition (WR) in 10% reverberation and WR in the presence of ipsilateral speech-weighted noise have been collected. These variables were correlated with OHC function measured by distortion-product otoacoustic emission (DPOAE) signal to-noise-ratio (SNR), and AN function measured by compound action potential (CAP) peak amplitude and ratio to summating potential measured using electrocochleography.

Synaptopathy, by this operational definition, may be present in as many as 30% of individuals with normal hearing. Persons hearing within normal limits may exhibit HIN difficulties, and persons with hearing within normal limits may exhibit two distinct types of otopathologies undetected by the standard audiogram (a.k.a. hidden hearing loss) namely operational cochlear synaptopathy and OHC dysfunction. AN untuning secondary to OHC dysfunction is a third otopathology that occurs in subjects with a Mild-Moderate sensorineural hearing loss (SNHL). Clinical norms for each of these otopathologies are presented. Finally, the data show that operational cochlear synaptopathy does not correlate with HIN dysfunction. Rather, HIN performance is primarily governed by OHC function, while AN untuning also plays a lesser but statistically significant role.

The results of this study suggest the following: (1) persons hearing within normal limits may exhibit HIN difficulties; (2) persons hearing within normal limits may exhibit undetected otopathologies, namely AN dysfunction and OHC dysfunction; (3) AN untuning secondary to OHC dysfunction occurs in subjects with Mild-Moderate SNHL; (4) HIN performance is primarily governed by OHC function rather than AN function.