Bilateral Sensorineural Hearing Loss and Polyneuropathy in Sweet’s Syndrome

Links checked 2/03/17.

This is a very interesting case-study reporting bilateral sensorineural hearing loss and polyneuropathy in a patient with Sweet’s syndrome (Cala et al, 2015).

A 54-year-old man went to clinic with ulcers on his fingers along with symptoms suggesting a neurological problem, e.g. numbness and pins and needles in the extremities (hands and feet). He also had rapidly progressive hearing loss. Four years earlier, he had been diagnosed with Sweet’s syndrome (SS) by another clinic after developing skin lesions involving all four extremities. He did not recall having any neurological symptoms at that time. A biopsy (tissue sample) of the lesions had shown a dense neutrophilic infiltrate (lots of white blood cells called neutrophils in the tissues) and an absence of vasculitis (no inflammation of the vessels). These biopsy results suggested SS, and the patient was started on oral prednisone and given steroid injections as necessary to treat any new lesions. Dapsone gel and silver sulfadiazine (an antibacterial agent) were also used to help treat the lesions and reduce the risk of infection. However, an ulcerated finger became badly infected and needed to be amputated.

Over the next few months, after his latest visit to the clinic, the patient started to develop new lesions on his skin. The numbness and pins and needles in his feet also started to get worse, and would later affect the hands. Cerebrospinal fluid analysis (CSF) showed a normal white blood cell count. This is usually raised in neuro-Sweet’s disease – a neurological variant of Sweet’s syndrome that affects the brain and spinal cord. A serum immunofixation electrophoresis blood test suggested that the patient had probably developed SS secondary to monoclonal gammopathy of unknown significance (MGUS). Other tests included nerve conduction and a nerve biopsy, and the results of these tests showed that the patient had developed polyneuropathy. This is a condition that affects the peripheral nerves in roughly the same areas on both sides of the body. It is caused by disease or damage to the nerves that lie outside of the central nervous system, i.e. the brain and spinal cord. The patient was then evaluated for his worsening hearing loss which was causing dizziness and tinnitus (‘ringing in the ears’ or other sounds). A hearing test known as an audiogram revealed serious sensorineural hearing loss in both ears, despite the fact that no abnormalities had been shown on a MRI scan. Sensorineural hearing loss is a form of hearing impairment that is caused by damage to the nerves in the inner ear.

It was eventually determined that all of the patient’s symptoms were probably caused by SS. After treatment with azathioprine and intravenous immunoglobulin (IVIg), his skin lesions started to settle, and his polyneuropathy also started to improve as a result of the IVIg. However, his hearing loss did not improve, even after additional treatment with mycophenolate mofetil, cyclosporine, pregabalin, gabapentin, and nortriptyline. Only after cochlear implants did some, but not all, of his hearing return.

Please note that the majority of Sweet’s syndrome patients will not develop hearing loss or polyneuropathy as a result of their condition.

References.

Cala, C. , Kole, L. and Sami, N. (2015) Bilateral Sensorineural Hearing Loss and Polyneuropathy in a Patient with Sweet’s Syndrome. Case Reports in Otolaryngology, Dec 6th (Hindawi).

Further information.

Action on Hearing Loss (2017) Cochlear Implants (online). Accessed 2/03/17.

Macmillan Cancer Support (2017) General information: MGUS (online). Accessed 2/03/17.

NHS Choices (2015) Hearing Loss – Causes (online). Last reviewed on 22/04/15. Includes information on sensorineural hearing loss.

NHS Choices (2016) Peripheral Neuropathy (online). Last reviewed on 16/05/16. Includes information on polyneuropathy.

What is the treatment for Sweet’s syndrome?

© 2012-2017 Sweet’s Syndrome UK

 

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