An unusual case of post-cochlear implant performance degradation in a patient with suspected Cogan’s syndrome

Hearing loss in inner ear and systemic autoimmune disease: A systematic review of post‐cochlear implantation outcomes

“…A minority of patients had primary AIED (38) compared to secondary AIED (77) such as Cogan's syndrome (n = 42), relapsing polychondritis (n = 6), ANCA‐associated vasculitis (n = 4), rheumatoid arthritis (n = 3), granulomatosis with polyangiitis (n = 3), inflammatory bowel disease (n = 2), Vogt‐Koyanagi‐Harada syndrome (n = 2), polyarteritis nodosa (n = 2), unspecified vasculitis (n = 2), eosinophilic granulomatosis with polyangiitis (n = 1), Beçet's disease (n = 1) cerebral vasculitis (n = 1), Sjögren's syndrome (n = 1), primary sclerosing cholangitis (n = 1), neurosarcoidosis (n = 1), systemic psoriasis (n = 1), systemic lupus erythematosus (n = 1), Sweet's disease (n = 1), chronic demyelinating inflammatory polyneuropathy (n = 1), and systemic sclerosis (n = 1). Diagnosis was mostly clinical, however one study 21 conducted genetic tests to rule out other causes (Muckle‐Wells syndrome). Common presenting symptoms included vestibular symptoms (26% of patients reporting dizziness, vertigo, or unsteadiness) and tinnitus (18%).…”

“…Kontorinis 25 similarly reported a case (Cogan's syndrome) with recurrent skin infections that was treated with antibiotics, and Low 31 reported a patient (Cogan's syndrome) with scalp pressure sore from the dressing that healed conservatively. Other reports not within the immediate post‐operative period (>6 months, or time not reported) include: CI failure (n = 2, one of which had Cogan's syndrome, and the other was not specified in a mixed group), 10,30 facial tactile sensations (n = 1, Cogan's syndrome), 27 and worsening facial pain with reduced hearing bilaterally (n = 1, Cogan's syndrome) 21 . The remainder of the studies did not state any surgical complications, and Bacciu 23 explicitly stated that none of their patients suffered from complications from their flap or systemic disease.…”

“…Theoretically, osteoneogenesis inside the cochlea could lead to an increase in electrical impedance over time, resulting in reduced CI efficiency and function. However, of the 85 patients (10 studies) in this review that were reported with consecutive audiometric data post‐operatively (or compared short term with long term follow‐up data), 30% 8,23,25,35 (26 patients) showed improvement in CI outcomes over a few years, 33% 11,27,42 (28 patients) reported patients with a “generally stable” hearing level over time, 35% 10,35,36 (30 patients) reported initial improvement up to 1 year and then plateauing or mild worsening of hearing thereafter, and 1.2% (1 patient) showed good initial response but complete deterioration due to pain after 18 months 21 . In one study, 11 a further sub‐group analysis suggested that cochlear implantation may initially show poor results in primary AIED, but then improve after 12 months; however this studies length of follow‐up (<2 years) may not be sufficient as symptomatic osteoneogenesis may be a lengthier process.…”

Scoping review of cochlear implantation in Susac's syndrome

“…Although the underlying pathophysiology is felt to differ, there are case reports of delayed CI performance degradation in patients with Cogan’s syndrome and relapsing polychondritis; still, stable objective hearing outcomes are achieved in the majority of these cases. 29 , 30 , 31 …”

“…While no previous cases of cochlear implantation in SS have noted fluctuating CI performance, our experience prompts consideration of additional transient central pathology that compromises implant performance. Although the underlying pathophysiology is felt to differ, there are case reports of delayed CI performance degradation in patients with Cogan's syndrome and relapsing polychondritis; still, stable objective hearing outcomes are achieved in the majority of these cases 29–31 …”