Incomplete Wolfram syndrome. Clinical case report

We describe clinical presentation of Wolfram syndrome and follow-up data in a child. Diagnostics of Wolfram syndrome takes time because clinical symptoms develop not at the time of disease manifestation, but usually several years later. The sequence of manifestations also varies. According to the literature, sensorineural hearing loss occurs in the 2nd decade, and bladder atony develops only by the 3rd decade. In the presented case, initial manifestations of bladder innervation disorders in the form of its dysfunction developed as early as the first year, and sensorineural hearing loss formed by the 4th year of the disease. As in other studies, the patient developed optic disc atrophy within the first year after diabetes onset. This clinical case confirms variability in the clinical symptoms of Wolfram syndrome. The sequence in which the disease picture develops (in this case, there was an incomplete form of syndrome the absence of diabetes insipidus) does not always coincide with the classic course of syndrome, which complicates timely diagnosis.

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Migraine increases the proportion of sudden sensorineural hearing loss: A longitudinal follow-up study

Auris Nasus Larynx ◽

10.1016/j.anl.2018.10.006 ◽

2019 ◽

Vol 46 (3) ◽

pp. 353-359 ◽

Cited By ~ 3

Author(s):

So Young Kim ◽

Mi Kyung Kim ◽

Jae-Sung Lim ◽

Il Gyu Kong ◽

Hyo Geun Choi

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Sudden Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Follow Up Study

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Unchanged unilateral hearing loss and ipsilateral growth of an acoustic neuroma from 1 to 4 cm

The Journal of Laryngology & Otology ◽

10.1017/s0022215100122704 ◽

1993 ◽

Vol 107 (3) ◽

pp. 230-232

Author(s):

Jos P. P. M. van Leeuwen ◽

Cor W. R. J. Cremers ◽

Henk O. M. Thijssen ◽

Henk E. Meyer

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

False Negative ◽

Pure Tone Audiometry ◽

Tumour Volume ◽

Sensorineural Hearing ◽

Speech Audiometry ◽

Volume Doubling Time ◽

Volume Doubling

Progressive sensorineural hearing loss is the most important early symptom of a cerebellopontine angle process. A case report is presented of a 42-year-old woman who was referred to our department in 1979. Oil cistemography showed non filling of the left internal acoustic canal. Audiometry was planned as the method of control, but she did not return until nine- years later. In 1988, an acoustic neurionoma of 4 cm diameter was found in the left CPA. Pure tone audiometry and speech audiometry showed that during the nine-year interval, her 60 dB flat sensorineural hearing loss and speech perception thresholds had remained almost unchanged. A follow-up with only tone and speech audiometry can lead to a false negative diagnosis in some of these cases. Calculation of the growth in tumour volume over nine years in this patient showed a tumour volume doubling time of about 15 months.

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A new semi-implantable middle ear implant for sensorineural hearing loss: three-years follow-up in a pilot patient’s group

Acta Oto-Laryngologica ◽

10.1080/00016489.2017.1371327 ◽

2017 ◽

Vol 138 (1) ◽

pp. 31-35 ◽

Cited By ~ 2

Author(s):

Maurizio Barbara ◽

Luigi Volpini ◽

Chiara Filippi ◽

Francesca Atturo ◽

Simonetta Monini

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Middle Ear ◽

Sensorineural Hearing ◽

Middle Ear Implant

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Third-generation bisphosphonates for cochlear otosclerosis stabilizes sensorineural hearing loss in long-term follow-up

Laryngoscope Investigative Otolaryngology ◽

10.1002/lio2.91 ◽

2017 ◽

Vol 2 (5) ◽

pp. 262-268 ◽

Cited By ~ 1

Author(s):

Taha A. Jan ◽

Aaron K. Remenschneider ◽

Christopher Halpin ◽

Margaret Seton ◽

Michael J. McKenna ◽

...

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Third Generation ◽

Long Term Follow Up

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Increased Risk of Sudden Sensorineural Hearing Loss in Patients With Osteoporosis: A Population-based, Propensity Score-matched, Longitudinal Follow-Up Study

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/jc.2014-4316 ◽

2015 ◽

Vol 100 (6) ◽

pp. 2413-2419 ◽

Cited By ~ 10

Author(s):

Mei-Chen Yeh ◽

Shih-Feng Weng ◽

Yuan-Chi Shen ◽

Chien-Wen Chou ◽

Chwen-Yi Yang ◽

...

Keyword(s):

Hearing Loss ◽

Propensity Score ◽

Sensorineural Hearing Loss ◽

Population Based ◽

Sudden Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Follow Up Study ◽

Increased Risk

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Metformin decreases the risk of sudden sensorineural hearing loss in patients with diabetes mellitus: A 14-year follow-up study

Diabetes and Vascular Disease Research ◽

10.1177/1479164119826292 ◽

2019 ◽

Vol 16 (4) ◽

pp. 324-327 ◽

Cited By ~ 4

Author(s):

Hsin-Chien Chen ◽

Chi-Hsiang Chung ◽

Chieh-Hua Lu ◽

Wu-Chien Chien

Keyword(s):

Diabetes Mellitus ◽

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Cox Regression ◽

Sudden Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Lower Percentage ◽

Research Database ◽

Patients With Diabetes

Background: The risk of sudden sensorineural hearing loss associated with metformin use in patients with diabetes mellitus has not been fully examined. Study design: It is a retrospective matched-cohort study. Subjects and methods: We examined the medical records of patients with diabetes mellitus over 18 years old in Taiwan’s National Health Insurance Research Database for the period between 1 January 2000 and 31 December 2013, to establish matched cohorts (14,109 with and 42,327 without metformin use) at a ratio of 1:3 by sex, age and index year. Results: We used a Cox regression hazard model to identify risk factors of sudden sensorineural hearing loss during 14 years of follow-up, and the results indicate that a significantly lower percentage of diabetes mellitus patients with metformin use ( p = 0.033) developed sudden sensorineural hearing loss compared with those without metformin use (0.21%, 29/14,109 vs 0.32%, 136/42,327). After adjustment for age and other variables [adjusted hazard ratio: 0.630 (95% confidence interval: 0.422–0.941, p = 0.024)], this study also demonstrated that metformin use appeared to reduce the risk of developing sudden sensorineural hearing loss. Conclusion: This study demonstrated an association between metformin use and lower incidence of sudden sensorineural hearing loss among patients with diabetes mellitus.

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Neurocognitive outcome in children with sensorineural hearing loss after treatment of malignant embryonal brain tumors.

Journal of Clinical Oncology ◽

10.1200/jco.2017.35.15_suppl.2029 ◽

2017 ◽

Vol 35 (15_suppl) ◽

pp. 2029-2029

Author(s):

Christine Dahl ◽

Iska Moxon-Emre ◽

Vijay Ramaswamy ◽

Ute Katharina Bartels ◽

Uri Tabori ◽

...

Keyword(s):

Hearing Loss ◽

Brain Tumors ◽

Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Verbal Language ◽

Intellectual Function ◽

Embryonal Brain ◽

Mild Hearing Loss ◽

The Impact

2029 Background: Neurological side effects associated with childhood brain tumors and their treatments contribute to long term neurocognitive morbidity. The aims of this study were to identify the incidence of sensorineural hearing loss (SNHL) in a large sample of children treated for malignant brain tumors, and to evaluate the potential relationship between SNHL and intellectual functioning following the completion of treatment. Methods: We conducted a prospective follow-up study at a single center with review of 119 patients treated for embryonal brain tumors at the Hospital for Sick Children, between 1996-2015, to analyze the impact of significant SNHL (Chang > 2b) on intellectual function. Hearing was assessed post-treatment (median age: 13.5y (+4.5)) and the median age for neurocognitive testing was 12.8y (+ 4.1). The median interval from time of diagnosis was 5.8y (+ 3.7). Results: Severe SNHL was identified in half the patients (50.4%, n = 60/119). We identified a subset of patients (n = 61) who had assessments of intellectual function. In this cohort, intellectual function was significantly poorer in the group with severe SNHL, even after controlling for the effect of craniospinal radiation (severe SNHL 22.4 Gy + 13.3, no or mild hearing loss 20.4 Gy +12.8) and boost dose and volume. Children experiencing severe SNHL had lower overall IQ (severe SNHL 72.4 + 16.6; no/mild hearing loss 92.0 + 20.5) p < 0.001 and in significantly lower verbal comprehension (severe SNHL 78.7 + 15.9; no/mild hearing loss 94.7 + 13.8) p < 0.001, and working memory (severe SNHL 78.2+ 17.6; no/mild hearing loss 94.8 + 16.4) p < 0.001, scores. Conclusions: Hearing loss is a much more significant complication in children with embryonal brain tumors than previously estimated. We show the profound impact of hearing loss on intellectual deficit in children. Namely, patients with severe SNHL have difficulty using and understanding verbal language, and they have a reduced ability to concentrate and manipulate information in short-term memory. Our results have implications on future trial designs and follow-up of children treated for embryonal brain tumors.

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The Short- and Long-Term Outcome of Intratympanic Steroid Therapy as a Salvage Treatment for Acute Low-Tone Sensorineural Hearing Loss without Episodes of Vertigo

Audiology and Neurotology ◽

10.1159/000444577 ◽

2016 ◽

Vol 21 (3) ◽

pp. 132-140 ◽

Cited By ~ 4

Author(s):

Shinya Morita ◽

Yuji Nakamaru ◽

Keishi Fujiwara ◽

Keiji Iizuka ◽

Masayori Masuya ◽

...

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Salvage Treatment ◽

Sensorineural Hearing ◽

Control Group ◽

Long Term Follow Up ◽

Low Tone ◽

Hearing Outcomes

Objectives: To evaluate the hearing outcomes of intratympanic steroid (ITS) treatment for patients with acute low-tone sensorineural hearing loss (ALHL) after failure of initial therapy and to investigate the recurrence and progression to definite Ménière's disease (MD) during a long-term follow-up. Methods: We retrospectively reviewed the medical records of 90 patients with refractory ALHL who were followed up for at least 1 year between January 2000 and April 2014. Patients who responded poorly to initial medical treatment received intratympanic dexamethasone injections (ITS group) or isosorbide administration for 4 weeks (diuretic group) as salvage treatment options according to their choice of management. The control group did not receive ITS or the diuretic, due to their refusal of both medical treatments. The hearing outcomes were evaluated 1 month, 1 year and 5 years after the completion of the second-line therapy, and the rates of recurrence and progression to MD were measured during a follow-up period of at least 1 year. Results: Twenty-seven patients in the ITS group, 39 patients in the diuretic group and 24 patients in the control group were enrolled. Of these, 12 patients in the ITS group, 15 patients in the diuretic group and 12 patients in the control group were followed up for over 5 years. We found that the recovery rates and the audiometric functional values after 1 month and 1 year in the ITS group were significantly higher than those in the diuretic and control groups. However, there were no significant differences in the recovery rates or the audiometric functional values after 5 years, or in the rates of recurrence and progression to MD between the groups. Conclusions: Salvage ITS therapy can provide a relatively good short-term hearing outcome for ALHL patients who have persistent hearing loss despite conventional treatment. However, both recurrence and progression to MD after treatment were observed in some patients during the long-term follow-up.

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