Selective bilateral vestibular neuropathy in a Turkish CMT1B family with a novel MPZ mutation

Purpose of reviewTo report findings in 12 members over 3 generations of a family with dominantly inherited Charcot Marie Tooth disease (CMT1B) due to a novel MPZ mutation, who all had moderately severe, selective impairment of vestibular function with normal hearing. Methods used were video head impulse testing of the function of all 6 semicircular canals, Romberg test on foam, nerve conduction studies, whole exome and Sanger sequencing.Recent findingsAll affected patients had a demyelinating neuropathy and a novel MPZ mutation: c.362A>G (chr1: 161276584, p.D121G). All also had normal hearing for age but a moderately severe impairment of semicircular canal function and a positive Romberg test on foam.SummarySome CMT mutations can impair vestibular function, presumably due a vestibular nerve involvement but spare hearing. In such patients impairment of vestibular function as well as impairment of proprioception contributes to imbalance.

Lindsay-Hemenway Syndrome: Review of the literature and case report

OBJECTIVES. Reviewing the literature data related to Lindsay – Hemenway syndrome.MATERIAL AND METHODS. We searched PubMed and Google Scholar with the key words of “Lindsay-Hemenway syndrome”, “benign positional vertigo”, “vestibular rehabilitation”RESULTS. Lindsay-Hemenway syndrome is characterized by an association between vestibular neuronitis and BPPV. The specificity of the syndrome consists in the existence of an initial episode of acute vestibular neuropathy manifested by intense vertigo and nystagmus, followed in a variable time frame by episodes of posterior canal BPPV. The treatment of the syndrome consists in a combination of otolith repositioning manoeuvres and vestibular rehabilitation therapy. The physicians involved in treating patients with vestibular disorders should be aware of the existence of this syndrome in order to diagnose and treat the patients accordingly.CONCLUSION. The Lindsay-Hemenway syndrome is a challenge for the physician. In order to establish a diagnosis, a careful investigation of clinical history and objective examination are needed. The clinician should take into consideration the presence of a sudden vertigo without deafness followed by postural nystagmus, and unilateral labyrinthine hyporeflexia or absence of reflectivity. For a successful therapeutic approach, we should be able to combine manoeuvres of repositioning for BPPV with an appropriate vestibular rehabilitation therapy in order to ensure a correct central compensation of the peripheral unilateral deficit.

Vestibular Neurectomy for Intractable Vertigo: Case Series and Evaluation of Role of Endoscopic Assistance in Retrolabyrinthine Craniotomy

Objective This study evaluates the utility of endoscopy for retrolabyrinthine vestibular nerve section (RLVNS). Design/Setting This is a retrospective review for RLVNSs by the senior author. The endoscope's utility was assessed and assigned a grade based on operative findings. Participants/Main Outcome Measures Fifteen patients (eight males and seven females; 53 and 47%, respectively) were identified with mean age 56.7 years. Indications included Ménière's disease (MD) in 12 of 15 patients (80%), uncompensated vestibular neuritis in 2 patients (13%), and other vestibular neuropathy in 1 patient (7%). Vertigo resolved in 14 of 15 patients (93%). Complications included decreased hearing in two patients (13%) and deep venous thrombosis in one patient (7%). There were no facial nerve complications or mortalities. Results Sectioning vestibular division of the vestibular–cochlear nerve was achieved without perceived benefit of endoscopy in the 80% of cases (grade 0, n = 12). Endoscopy was helpful in patients with a small mastoid (grade 1, n = 2, 13.3%), and deemed necessary where the flocculus of the cerebellum was adherent to the eighth nerve arachnoid at the porus acusticus (grade 2, n = 1, 6.7%). Conclusion RLVNS is a safe and efficacious procedure for the treatment of vertigo; the surgical endoscope may be a useful adjunct in selected cases. Patients with MD may expect the greatest benefit from surgery.