By Marcello Cherchi, MD PhD
For patients
The phrase “sympathetic ear disease” refers to a situation in which one ear has been damaged, and after some delay, the other ear develops symptoms. This is usually attributed to the immune system being erroneously activated and then mis-directing antibodies at a healthy organ.
For clinicians
Overview
Sympathetic ear disease is a rare condition thought to result when antigens (of the labyrinth or vestibular nerve) are exposed to the immune system (secondary to disease, trauma or an invasive procedure), after which the immune system mounts an attack against the contralateral (previously healthy) ear, typically manifesting as sensorineural hearing loss. Immunosuppression seems like a logical approach in this scenario, but has not been formally studied.
Details
Sympathetic ear disease is rare. In simple terms, sympathetic ear disease can happen when one ear suffers an insult, and then after some delay the other ear develops symptoms – typically sensorineural hearing loss. Sympathetic ear disease has been reported contralateral to an ear affected by diseases such as vestibular schwannoma (Early et al. 2020) and temporal bone fracture (ten Cate and Bachor 2005). It has also been reported contralateral to an ear that has undergone an invasive procedure, such as after surgery for vestibular schwannoma (Clemis, Mastricola, Schuler-Vogler 1982; Harris, Low, House 1985), endolymphatic sac decompression (Ochoa and Weider 2003) and stapedectomy revision (Richards, Moorhead, Antonelli 2002).
The hypothesized mechanism of sympathetic ear disease is that trauma or some other disease disrupts the blood-labyrinth barrier (Nyberg et al. 2019), which gives the immune system access to labyrinthine antigens, which may in turn trigger the immune system to mount a mis-directed attack at the other (previously healthy) ear. Such immune attacks can happen at some delay after the original insult, ranging from months to years.
Sympathetic ear disease is thought to be the otologic analog of comparable phenomena such as sympathetic ophthalmia (Parchand et al. 2022). Given the immune-mediated nature of sympathetic ophthalmia, treatment is often attempted with corticosteroids or other immunosuppressants.
While immunosuppression seems logical, there are no trials of this approach in the treatment of sympathetic ear disease.
References
Clemis JD, Mastricola PG, Schuler-Vogler M (1982) Sudden hearing loss in the contralateral ear in postoperative acoustic tumor: three case reports. Laryngoscope 92: 76-9.
Early S, Rinnooy Kan CE, Eggink M, Frijns JHM, Stankovic KM (2020) Progression of Contralateral Hearing Loss in Patients With Sporadic Vestibular Schwannoma. Front Neurol 11: 796. doi: 10.3389/fneur.2020.00796
Harris JP, Low NC, House WF (1985) Contralateral hearing loss following inner ear injury: sympathetic cochleolabyrinthitis? Am J Otol 6: 371-7.
Nyberg S, Abbott NJ, Shi X, Steyger PS, Dabdoub A (2019) Delivery of therapeutics to the inner ear: The challenge of the blood-labyrinth barrier. Sci Transl Med 11. doi: 10.1126/scitranslmed.aao0935
Ochoa VM, Weider DJ (2003) Development of autoimmune sensorineural hearing loss after endolymphatic sac decompression: two case reports. Otol Neurotol 24: 279-82.
Parchand S, Agrawal D, Ayyadurai N, Agarwal A, Gangwe A, Behera S, Bhatia P, Mulkutkar S, Barwar G, Singh R, Sen A, Agarwal M (2022) Sympathetic ophthalmia: A comprehensive update. Indian J Ophthalmol 70: 1931-1944. doi: 10.4103/ijo.IJO_2363_21
Richards ML, Moorhead JE, Antonelli PJ (2002) Sympathetic cochleolabyrinthitis in revision stapedectomy surgery. Otolaryngol Head Neck Surg 126: 273-80. doi: 10.1067/mhn.2002.122702
ten Cate WJ, Bachor E (2005) Autoimmune-mediated sympathetic hearing loss: a case report. Otol Neurotol 26: 161-5.
