Review Question - QID 221232

This older patient with slowly progressive bilateral sensorineural hearing loss that is more pronounced at higher frequencies most likely has presbycusis.

Presbycusis is an age-related, sensorineural hearing loss that results from damage to the cochlear hair cells and the spiral ganglion cells in the vestibulocochlear nerves. It is the most common cause of sensorineural hearing loss and is present in over half of patients by the age of 65 years. It manifests with progressive bilateral hearing loss that is most pronounced at higher frequencies. Hearing deficits are exacerbated in the setting of background noise, as higher frequencies allow the inner ear to better focus on sounds of interest. Constant tinnitus and vestibular end-organ dysfunction may also manifest later in the disease course. Evaluation should include otoscopic examination and a formal audiogram. Although there is no directed therapy for presbycusis, symptomatic management can include assistive listening devices, hearing aids, and cochlear implantation.

Cunningham and Tucci review the evidence regarding hearing loss in adults. They discuss how presbycusis is a very common cause of hearing loss. They recommend treatment with assistive devices.

Figure/Illustration A is an audiogram showing decreased hearing in the high frequency ranges (red circle). This type of hearing loss is seen in patients with presbycusis.

Incorrect Answers:
Answer 1: Acoustic neuromas are Schwann cell-derived tumors that commonly affect the vestibular portion of cranial nerve 8. Although they present with sensorineural hearing loss that is more pronounced at higher frequencies, acoustic neuromas typically cause asymmetric hearing loss. Patients with neurofibromatosis type 2 can have bilateral acoustic neuromas; however, these patients typically present earlier in life.

Answer 2: Cholesteatoma is a keratinized, desquamated epithelial collection in the middle ear that is often a sequela of chronic otitis media. Conductive hearing loss usually occurs late in the course of the disease. In addition, cholesteatomas can often be visualized directly on otoscopic exam.

Answer 3: Jervell and Lange-Nielsen Syndrome is a potassium channelopathy that causes congenital sensorineural hearing loss and prolonged QTc intervals. It would not be expected to cause progressive hearing loss in an older adult.

Answer 4: Otosclerosis is a bony overgrowth in the footplate of the stapes that inhibits acoustic transduction in the middle ear. It causes conductive, not sensorineural, hearing loss that is more pronounced at lower frequencies. Rinne testing would show bone conduction greater than air conduction, and Weber testing would localize to the more affected ear.

Bullet Summary:
Presbycusis is a sensorineural loss that typically occurs in older adults and presents with difficulty hearing high frequencies.