Sensorineural deafness

Symptoms may include:

• Some sounds seem overly loud in one ear.
• You have problems following conversations when two or more people are talking.
• You have problems hearing in noisy areas.
• It is easier to hear men's voices than women's voices.
• It is hard to tell high-pitched sounds (such as "s" or "th") from one another.
• Other people's voices sound mumbled or slurred.
• You have problems hearing when there is background noise.

Associated symptoms may include:

• Feeling of being off-balance or dizzy (more common with Meniere disease and acoustic neuromas)
• Ringing or buzzing sound in the ears (tinnitus)

The inner part of the ear contains tiny hair cells (nerve endings), that change sounds into electric signals. The nerves then carry these signals to the brain.

Sensorineural hearing loss (SNHL) is caused by damage to these special cells, or to the nerve fibers in the inner ear. Sometimes, the hearing loss is caused by damage to the nerve that carries the signals to the brain.

Sensorineural deafness that is present at birth (congenital) is most often due to:

• Genetic syndromes
• Infections that the mother passes to her baby in the womb (toxoplasmosis, rubella, herpes)

SNHL may develop in children or adults later in life (acquired) as a result of:

• Age-related hearing loss
• Disease of the blood vessels
• Immune disease
• Infections, such as meningitis, mumps, scarlet fever, and measles
• Injury of the ear or head
• Loud noises or sounds, or loud sounds that last for a long time
• Meniere disease
• Tumor, such as acoustic neuroma
• Use of certain medicines
• Working around loud noises every day

In some cases, the cause is unknown.

The goal of treatment is to improve your hearing. The following may be helpful:

• Hearing aids
• Telephone amplifiers and other assistive devices
• Safety and alert systems for your home
  
• Sign language (for those with severe hearing loss)
• Speech reading (such as lip reading and using visual cues to aid communication)

A cochlear implant may be recommended for certain people with severe hearing loss. Surgery is done to place the implant. The implant makes sounds seem louder, but does not restore normal hearing.

You will also learn strategies for living with hearing loss and advice to share with those around you for talking to someone with hearing loss.

Nerve deafness; Hearing loss - sensorineural; Acquired hearing loss; SNHL; Noise-induced hearing loss; NIHL; Presbycusis

Arts HA, Adams ME. Sensorineural hearing loss in adults. In: Flint PW, Francis HW, Haughey BH, et al, eds. Cummings Otolaryngology: Head and Neck Surgery. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 152.

Eggermont JJ. Types of hearing loss. In: Eggermont JJ, ed. Hearing Loss. Cambridge, MA: Elsevier Academic Press; 2017:chap 5.

Le Prell CG. Noise-induced hearing loss. In: Flint PW, Francis HW, Haughey BH, et al, eds. Cummings Otolaryngology: Head and Neck Surgery. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 154.

National Institute on Deafness and Other Communication Disorders website. Noise-induced hearing loss. NIH Pub. No. 14-4233. www.nidcd.nih.gov/health/noise-induced-hearing-loss. Updated March 16, 2022. Accessed May 8, 2024.

Shearer AE, Shibata SB, Smith RJH. Genetic sensorineural hearing loss. In: Flint PW, Francis HW, Haughey BH, et al, eds. Cummings Otolaryngology: Head and Neck Surgery. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 150.

Updated by: Josef Shargorodsky, MD, MPH, Johns Hopkins University School of Medicine, Baltimore, MD. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.