When It Rains It Pours...: Wernicke Encephalopathy with Sudden Blindness after Bariatric Surgery in a Patient with Congenital Deafness.

BACKGROUND: Malabsorption syndromes are known chronic complications of bariatric surgery. Therefore, it is recommended to take oral supplementation with multivitamins. Wernicke's encephalopathy represents an acute neuropsychiatric syndrome associated with alcoholism or severe malnutrition; sporadic cases of this potential complication related to bariatric surgery are described in the literature. We present a case of Wernicke's encephalopathy due to severe vitamin B1 deficiency after bariatric surgery. CASE REPORT: A 31-year-old woman with deaf-mutism from the age of 3 years old, operated 3 months before with a mini-gastric bypass for severe obesity, was transferred to our unit after accessing the emergency room. In the immediate medical history, there was the sudden and rapid decline in vision, leading to complete loss of vision, marked asthenia, and paresthesia in the four limbs. Considering the previous bariatric surgery, the diagnosis of non-alcoholic Wernicke's syndrome was suspected, for which IV therapy with Vitamin B1 was started at a dosage of 5 vials of 200 mg in 100 cc of saline solution (three times a day for the first 72 hours, subsequently 1 once/day). After 12 hours, there was an improvement in visual acuity, and the symptoms completely resolved within 48 hours. She was discharged with complete resolution of all symptoms after 1 month. CONCLUSION: Initial vision loss without confusion or encephalopathy is one atypical presentation of Wernicke syndrome. Clinical suspicion must be high in case of alcoholism or post-bariatric surgery. Early recognition of atypical symptoms, including vision loss, and timely administration of therapy improves the prognosis of this potentially reversible but time-dependent neurological emergency.

The Inheritance of Hearing Loss and Deafness: A Historical Perspective.

If the term "genetics" is a relatively recent proposition, introduced in 1905 by English biologist William Bateson, who rediscovered and spread in the scientific community Mendel's principles of inheritance, since the dawn of human civilization the influence of heredity has been recognized, especially in agricultural crops and animal breeding. And, later, in familial dynasties. In this concise review, we outline the evolution of the idea of hereditary hearing loss, up to the current knowledge of molecular genetics and epigenetics.

Imaging in congenital inner ear malformations-An algorithmic approach.

Malformations of the inner ear are an important cause of congenital deaf-mutism. Arrest in embryologic development of inner ear during various stages gives rise to the variety of malformations encountered. Current treatment options include hearing aids, cochlear implants, and auditory brainstem implants (ABI). With the advent of cochlear implant surgery and ABI, decent functional outcomes can be obtained provided such cases are diagnosed correctly and timely. To that end, high-resolution computed tomography (HRCT) has a fundamental role in the assessment of these conditions, ably supplemented by magnetic resonance imaging (MRI). The purpose of this pictorial essay is to illustrate the imaging features of inner ear anomalies in children with congenital deaf-mutism as per the latest terminology and classification and provide an algorithmic approach for their diagnosis.

The demographic work of Sir William Wilde.

This paper argues that Sir William Wilde was indeed a pioneering demographer. It also describes the unveiling of the plaque commemorating Sir William Wilde at his home, 1, Merrion Square, Dublin on the 28 October 1971.

Technical survey of the French role in multichannel cochlear implant development.

The objective of this review is to remind the ENT community of the essential role of the French teams in the development and finalization of the multi-electrode cochlear implant (MCI), which has deliberately been neglected, and to repair the oblivion into which France was curiously cast at the end of the last century. It aims to underline significant scientific publications from the researchers who played key roles in the development of MCIs. In conclusion, the Parisian team of the ENT Lab in Saint Antoine Hospital in Paris can claim priority for its work in five regards. We were the first: (1) to plot in 1976 a frequency map of the whole length of three living human cochleas; (2) on September 22, 1976, to set up total cochlear implantation in a deaf adult male with eight electrodes; (3) on March 16, 1977, to apply for a patent for an implantable hearing aid in humans; (4) to describe sound signal processing (SSP) for a functional cochlear implant able to supply totally deaf patients with speech discrimination without the help of lip-reading; (5) in 1983, to experimentally demonstrate why it was necessary to place a cochlear implant as early as possible, in case of profound neonatal deafness. An injustice has occurred. These facts will be brought to the knowledge of the scientific community.

Recollections of J.B.S. Haldane, with special reference to Human Genetics in India.

This paper is a brief account of the scientific work of J.B.S. Haldane (1892-1964), with special reference to early research in Human Genetics. Brief descriptions of Haldane's background, his important contributions to the foundations of human genetics, his move to India from Great Britain and the research carried out in Human Genetics in India under his direction are outlined. Population genetic research on Y-linkage in man, inbreeding, color blindness and other aspects are described.

Erythrokeratodermia Variabilis with Congenital Deaf-Mutism / 대한피부과학회지

Erythrokeratodermia variabilis is a rare genodermatosis characterized by the coexistence of randomly occurring, transient, erythematous patches and hyperkeratosis of the skin. A 25-year-old Uzbekistanian female with congenital deaf-mutism presented with well demarcated geographic hyperkeratotic patches and bizarre erythema on the whole body surface except the face, hands and feet from birth. The histopathological examination revealed marked hyperkeratosis, moderate acanthosis in the epidermis and vascular dilatation with a perivascular infiltration of inflammatory cells in the upper dermis. Herein we report on a rare case of erythrokeratodermia variabilis with congenital deaf-mutism which was rapidly improved by administering acitretin.

Epidemilogical profile of speech and language disorder in north central Nigeria.

BACKGROUND: Speech-language pathologists/Otolaryngologists recognize high prevalence of speech and language disorder among children. The aim of the study is to find out the epidemiological profile of speech and language disorder in north central Nigeria. METHOD: A five year retrospective review of all referral to speech and language therapy unit ENT Department between January 2005 and December 2009. Information retrieved and analysed included bio-data, clinical presentation and diagnosis of the patients. Descriptive analysis of these data were done. RESULTS: A total of 146 patients were seen out of which 89 (61%) were under five, 32 (21.9%) were between 5-10 years, 20 (13.7%) in the range of 11-20 yrs and 2 (1.4%) were between 21-34 yrs. None was observed in the elderly. Male preponderance was noted with male to female ratio of 1.9:1.0. The commonest diagnosis was deaf-mutism in 84 (57.5%) followed by delayed speech development in 31(21.2%) patients. The least diagnosis was aphasia in 2 (1.4%) patients. CONCLUSION: Speech and language disorder was commonest among the under five's with non in the elderly, There was a higher prevalence amongst males and deaf-mutism was the commonest observed.

Occlusal characteristics of deaf-mute individuals in the Turkish population.

OBJECTIVES: To classify and determine the occlusal characteristics of deaf-mute individuals and its gender distribution in the Turkish population. METHODS: For this study, 213 deaf-mute individuals (155 boys and 58 girls) were evaluated. The age range was between 10-24 years, and the mean age was 16.37+/-2.53 years. Measurements were divided into four groups: dental, intraarch, interarch, and, extra data. RESULTS: Of the participants, 75.0% had a Class I molar relationship, whereas 13.0% and 8.0% had Class II and Class III malocclusions, respectively. 23.9% of individuals had a normal overbite, 38.4% had a deepbite, and 23.4% had an openbite. One or more congenitally missing teeth were found in 6.0% of individuals; 81.0% expressed satisfaction with their esthetics, and 19.0% expressed the contrary. CONCLUSIONS: Different characteristics and malocclusions are present in deaf-mute individuals.

Cochlear Implant : Anaesthesia Challenges.

BACKGROUND: Cochlear implants are now an acceptable therapeutic option for those patients with irreversible hearing loss and deaf-mutism. The surgery is time consuming and complicated. Hence, the technique of anaesthesia plays a crucial role in success of cochlear implant surgery. Cochlear implant patients have various types of syndromes which are important from anaesthetic as well as surgical point of view. Pre-implant preparation requires objective assessment of hearing, plain X-rays of skull and a CT scan of the temporal bone. Anaesthesia is required for objective assessment of hearing in children under five years of age, to obtain X-rays of skull, magnetic resonance imaging (MRI), CT scan, brain evoked response audiometry (BERA) testing and finally for cochlear implant surgery. Anaesthetic considerations include preoperative familiarisation with the patient and his family. Syndromal illnesses have specific anaesthetic significance such as presence of difficult airway or prolonged QT interval. Parental presence is highly desirable during induction of anaesthesia. Electro-surgical instruments especially monopolar ones, should not be used once the cochlear implant is in place. METHODS: In our institution, all cases (15 children) received their cochlear implants under general anaesthesia and formed the study group. Children were administered midazolam orally and inj ketamine during CT scan. Our techniques of general anaesthesia were modified to permit use of nerve stimulators during surgery. To minimise the incidence of vertigo particularly after cochleostomy, postoperative nausea and vomiting, all patients received glycopyrrolate and fentanyl citrate intravenously prior to induction. Other agents used were, thiopentone, suxamethonium and end-tidal 1.3 MAC halothane in 1:2 mixture of oxygen and nitrous oxide (O2 and N2O). Electro diathermy was switched off, before the cochlear device was implanted on the patient. Patients were allowed to breathe spontaneously whenever nerve stimulator was used to locate the facial nerve. Inj Ondansetron 0.1 mg/kg was used as anti-emetic agent. Postoperative pain relief was initially provided with fentanyl 1 µg/kg IV and later with syrup ibuprofen. RESULTS: There were no remarkable anaesthetic or surgical complication in our series except perilymph leakage in two cases.