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|LETTER TO EDITOR
|Year : 2021 | Volume
| Issue : 6 | Page : 1893-1894
Neurobrucellosis—Overlooked but Treatable Cause of Hearing Loss
Waseem Dar, Maqbool Wani, Arjimand Yaqoob, Adnan Raina, Hilal Ganie, Zahoor Parray, Amit Chandra, Ravouf Asimi
Department of Neurology, Sheri Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India
|Date of Submission||22-Dec-2020|
|Date of Decision||23-Dec-2020|
|Date of Acceptance||29-Jan-2021|
|Date of Web Publication||23-Dec-2021|
Dr. Waseem Dar
Post Doc Fellow, Department of Neurology, Sheri Kashmir Institute of Medical Sciences, Srinagar - 190 011, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Dar W, Wani M, Yaqoob A, Raina A, Ganie H, Parray Z, Chandra A, Asimi R. Neurobrucellosis—Overlooked but Treatable Cause of Hearing Loss. Neurol India 2021;69:1893-4
Here we present two cases of hearing loss secondary to neurobrucellosis who remained undiagnosed till their hearing impairment became permanent and they developed other manifestations of the disease.
Our first case was a 20-year-old male patient admitted to our department with complaints of bilateral hearing loss for the past one year and weakness of lower limbs of two months duration. One year back patient had developed difficulty in hearing on left side which after a month also involved right ear. The hearing loss became profound within two months such that he could communicate with family members through writing only. He visited multiple otologists who did pure tone audiometery that was suggestive of bilateral profound sensorineural hearing loss. He received a cocktail of many medicines including steroids, multivitamins, and even antipsychotics and was finally planned for cochlear implant that was refused by attendants in view of financial constraints. Two months back patient developed weakness in his lower limbs in the form of difficulty in getting from sitting position and difficulty walking and required support of two persons for same. He reported no weakness in upper limbs, numbness, parasthesias, incontinence of urine or diplopia. On interviewing further, patient gave history of fever for the past 1 year that would come for a week then go for few weeks and relapse again; both the patient and physicians had ignored this fever. On examination, there was no fever, rash or lymphadenopathy. Neurological examination was remarkable for bilateral hearing loss, generalized hyporeflexia, and atrophy of thenar and hypothenar eminences of both hands. Muscle power was grade 4 in upper limbs and grade 3 in lower limbs. Sensory examination was normal and there was no sensory level. However, there was bilateral extensor plantar response. Baseline investigations were normal. Cerebrospinal fluid (CSF) analysis revealed lymphocytic pleocytosis (Total Leucocyte Count 200 cells/μl) with high protein (760 mg/dL) with low sugar (CSF sugar/blood sugar = 0.1). MRI imaging of patient revealed basal exudates with meningeal enhancement extending from brain to spine [Figure 1]. Brain stem auditory response showed no waveform recordable bilaterally while as visual evoked potential was normal. Nerve conduction studies revealed axonal neuropathy with absent F waves. A diagnosis of chronic meningitis with VIII nerve involvement and myeloradiculoneuropathy was made with diagnostic possibilities of infectious vs inflammatory disorders. However, CSF was more in the favour of chronic infections. Besides ANA, cANCA, pANCA, serum and CSF ACE levels, X-ray chest, urine examination, kidney function tests, and ultrasound abdomen were normal ruling out sarcoidosis or vasculitis. Meanwhile, his other CSF studies became available which revealed high CSF titers of Brucella antibodies (1:320) with serum titers of 1:20. Tubercular and fungal studies were negative. A diagnosis of neurobrucellosis presenting as Chronic Meningitis with myeloradiculoneuropathy and VIII nerve involvement was made and patient treated with a regimen of rifampicin, doxycycline, and ceftriaxone. He has completed five weeks of treatment and his weakness has improved significantly although there is only minimal improvement in hearing loss.
|Figure 1: T1 MRI with contrast showing basal exudates and leptomeningeal enhancement in the brain as well as the spinal cord|
Click here to view
Our second case was a 36-year-old female admitted to our department with complaints of fever, headache with vomiting, and encephalopathy of ten days duration. She had significant past history however; she had developed progressive hearing loss one year back in both her ears for which she had gone to ENT specialists and finally received a cochlear implant. On examination, she was febrile and stuporus. She had neck stiffness but other neurological examination was normal except for bilateral hearing loss for which she was on cochlear implant. A diagnosis of acute meningoencephalitis was made. CSF analysis showed neutrophilic pleocytosis (240 cells/μl), with high protein (221 mg/dl) and low sugar (CSF sugar/blood sugar = 0.12). A non-contrast CT scan of brain revealed hydrocephalus. Her CSF studies were negative for gram stain, neurotrophic viral panel. AFB stain, fungal studies and PCR for mycobacterium tuberculosis. However, her CSF Brucella antibody titers (1:320) and serum Brucella titers ((1:640) were high. A diagnosis of neurobrucellosis presenting as acute meningoencephalitis was made and patient treated with a regimen of ceftriaxone, rifampicin, and doxycycline. Patient became afebrile in hospital and her altered sensorium improved. She has completed five weeks of treatment and is doing well on our follow-up.
Brucellosis is the most common zoonotic disease worldwide affecting half a million people annually worldwide. It is an important, but underdiagnosed, disease in India where more than 80% population is rural and have contact with domesticated animals. Besides brucellosis is a great masquerader, often confused with tuberculosis. Brucellosis often presents as a chronic febrile illness with profound musculoskeletal symptoms. The fever in brucellosis is undulating in nature, meaning it comes for a week or so and then disappears for few weeks only to relapse again. In our first patient, fever being undulating in pattern was ignored by patient as well as the physician and hence delayed the diagnosis. In our second patient hearing loss was not evaluated initially and could have been because of neurobrucellosis although this can't be ascertained. Although patients with brucellosis often have misery, depression and are lethargy, neurological involvement is rare only reported in 0.2-5% cases. Chronic meningitis is the most reported nervous system manifestation of the disease, often associated with involvement of cranial nerve VIII. Although the cause for the specificity of 8th cranial nerve involvement in neurobrucellosis is not known, direct injury of the nerve by Brucella endotoxin has been proposed. Other cranial nerves like facial nerve, optic nerve and abducens nerve are less commonly involved. Although clinically significant hearing loss in brucellosis may be less common (9-16%), unapparent hearing loss detected by pure tone audiometery is very common particularly at higher frequencies and it improves with treatment. Most of the brucellosis patients with hearing loss also have other manifestations of the disease making diagnosis little easier; however, isolated hearing loss secondary to Brucella infection has been reported. Early diagnosis is paramount to successful treatment as patients may be left with permanent hearing loss., Such patients may then need cochlear implant to improve communication. Both of our patients had isolated hearing loss on initial presentation although one patient also had fever which due to undulating nature was ignored both by the physician and the patient. Such patients if picked early could have a better outcome of hearing loss which was unfortunately not possible in our patients. We thus recommend Brucellosis as a differential diagnosis in unexplained hearing loss as Brucellosis is endemic but underdiagnosed in India. Evaluation should include serological tests of CSF as well as serum for Brucella as well cultures of CSF and blood as these investigations have low sensitivity for detection of brucellosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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