Superficial siderosis of the central nervous system: phenotype and implications for audiology and otology

Superficial siderosis of the central nervous system (SSCN) results after chronic subarachnoid hemorrhage. Consequent demyelination, particularly of the cochleovestibular nerve and cerebellum, causes auditory-vestibular dysfunction. Predominant symptoms include progressive sensorineural hearing loss, imbalance, and ataxia. Despite characteristic auditory-vestibular involvement, SSCN is not well known among the hearing health community.

Clinical records of 49 patients diagnosed with SSCN were reviewed. Analysis included review of demographic, audiometric, and vestibular data of the largest sample to date and comparison to 31 audiovestibular case reports in the literature.

Hearing loss and disordered balance were reported by 92% and 67% of patients, respectively. Results suggest variable but substantial auditory-vestibular involvement related to SSCN. Hearing loss is typically progressive, sloping, and asymmetric and exceeds hearing loss expected based on age or sex. Decreased word recognition is possible and traditional amplification may fail to provide benefit.

SSCN is a destructive disorder affecting the auditory-vestibular system. Although not a common diagnosis, SSCN may be more prevalent than clinicians realize. Site of lesion may be anywhere within the auditory-vestibular system from the inner ear to the cortex, although the cochleovestibular nerve and cerebellum are particularly vulnerable. The progressive retrocochlear nature of the disorder makes differential diagnosis difficult and development of effective treatment options challenging. It is essential that audiologists and otologists recognize this uncommon cause of sensorineural hearing loss and balance disorder and the implications for evaluation, treatment, and counseling.