This conference is intended for otologists, audiologists, psychologists, hearing aid specialists, and nurses who provide clinical management services for patients with tinnitus. The conference will also provide information to patients who have tinnitus, their family, and friends.
Silence Is Bliss With a Few Home Adjustments
For hyperacusis patients, or people who have a sensitivity to everyday sounds, common household sounds can be a significant challenge, whether it’s a door closing or cooking a meal.
In Memoriam: Bryan Pollard of Hyperacusis Research
Bryan Pollard single-handedly created an entirely new diagnosis in the field of otology—pain hyperacusis—and worked tirelessly on behalf of those who suffered from it. He would become the most prominent patient-activist and the driving force for promoting research nationally focused on this condition.
The Gene C1ql1 Is Expressed in Adult Outer Hair Cells of the Mouse Cochlea
We found C1QL1 expression in the cochlear tissue of adult mice, but not in neonatal or developing mice, indicating that the protein is not involved with the development of any aspect of the auditory system. This developmental regulation is surprising as both C1QL1 and the related C1QL3 have synaptogenic functions.
Deciphering a Mechanism Behind Bone Conduction Hyperacusis
The underlying mechanism of bone conduction sound transmission to the inner ear has been elusive and poorly understood because bone conduction sound transmission is complex—multiple frequency-dependent mechanisms may be involved.
Investigating the Interaction of Auditory and Pain Pathways
As the intensity of a sound increases, typical-hearing listeners experience an increase in loudness, but for levels above 120 decibels (dB), listeners not only perceive the sound as extremely loud, but also painful—the aural threshold of pain. Some individuals with hearing loss and other neurological disorders perceive even moderate-intensity sounds as both painful and loud, a condition known as pain hyperacusis.
Advocating for Relief from Noise
When I was 8 years old, an audiologist declared that I have hyperacusis, a rare hearing condition that makes noise unbearable with no available cure. Eight years later, loudness is still completely intolerable for me, and I am committed to improving the lives of everyone in my situation through online education and advocacy.
Validating an Animal Model of Hyperacusis
To learn what is happening in the brain and nervous system when hyperacusis is present, we used sound-evoked, functional magnetic resonance imaging (fMRI) to locate regions of abnormal activity in the central nervous system of rats with behavioral evidence of hyperacusis induced with an ototoxic drug (sodium salicylate). We observed enhanced central auditory gain and were able to confirm this electrophysiologically.
Ears On Fire
A noise injury worsens readily. For hyperacusis sufferers such as myself, quiet makes the condition better; noise makes it worse. Among sufferers this is indisputable, but medical practitioners bizarrely treat quiet as harmful.
Quantifying the Effects of a Hyperacusis Treatment
By Xiying Guan, Ph.D
A typical inner ear has two mobile windows: the oval and round window (RW). The flexible, membrane-covered RW allows fluid in the cochlea to move as the oval window vibrates in response to movement from the stapes bone during sound stimulation.
Superior canal dehiscence (SCD), a pathological opening in the bony wall of the superior semicircular canal, forms a third window of the inner ear. This structural anomaly results in various auditory and vestibular symptoms. One common symptom is increased sensitivity to self-generated sounds or external vibrations, such as hearing one’s own pulse, neck and joint movement, and even eye movement. This hypersensitive hearing associated with SCD has been termed conductive hyperacusis.
Recently, surgically stiffening the RW is emerging as a treatment for hyperacusis in patients with and without SCD. However, the postsurgical results are mixed: Some patients experience improvement while others complain of worsening symptoms and have asked to reverse the RW treatment. Although this “experimental” surgical treatment for hyperacusis is increasingly reported, its efficacy has not been studied scientifically.
In the present study, we experimentally tested how RW reinforcement affects air-conduction sound transmission in the typical ear (that is, without a SCD). We measured the sound pressures in two cochlear fluid-filled cavities—the scala vestibuli (assigned the value “Psv”) and the scala tympani (“Pst”)—together with the stapes velocity in response to sound at the ear canal. We estimated hearing ability based on a formula for the “cochlear input drive” (Pdiff = Psv – Pst) before and after RW reinforcement in a human cadaveric ear.
We found that RW reinforcement can affect the cochlear input drive in unexpected ways. At very low frequencies, below 200 Hz, it resulted in a reduced stapes motion but an increase in the cochlear input drive that would be consistent with improved hearing. At 200 to 1,000 Hz, the stapes motion and input drive both were slightly decreased. Above 1,000 Hz stiffening the RW had no effect.
The results suggest that RW reinforcement has the potential to worsen low-frequency hyperacusis while causing some hearing loss in the mid-frequencies. Although this preliminary study shows that the RW treatment does not have much effect on air-conduction hearing, the effect on bone-conduction hearing is unknown and is one of our future areas for experimentation.
A 2017 ERG scientist funded by Hyperacusis Research Ltd., Xiying Guan, Ph.D., is a postdoctoral fellow at Massachusetts Eye and Ear, Harvard Medical School, in Boston.
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