Fluctuating mixed-type hearing loss associated with synovitis–acne–pustulosis–hyperostosis–osteomyelitis (SAPHO) syndrome

2010 ◽  
Vol 37 (4) ◽  
pp. 508-510 ◽  
Author(s):  
Shigetaka Shimizu ◽  
Kumiko Yukawa ◽  
Sachie Kawaguchi ◽  
Yukari Okubo ◽  
Mamoru Suzuki
Keyword(s):  
Hearing Loss ◽  
Mixed Type ◽  
Sapho Syndrome

Evaluation of Hearing and Balance Functions of Patients with Sickle Cell Anemia

2018 ◽  
Vol 23 (2) ◽  
pp. 122-125 ◽  
Author(s):  
Elif Tugba Sarac ◽  
Bilgehan Boke ◽  
Semsettin Okuyucu
Keyword(s):  
Hearing Loss ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Pure Tone ◽  
Mixed Type ◽  
Normal Hearing ◽  
Spontaneous Nystagmus ◽  
Control Group ◽  
Hearing Thresholds ◽  
Significant Difference

Introduction: Sickle cell anemia is a disease characterized by a wide vaso-occlusive incident from micro-vascular incident to muscularactivity. The cochlear function can also get affected by this vaso-occlusion. Objective: It is aimed at determining what kind of effects sickle cell anemia has on hearing and balance system. Methods: This study has been conducted on 46 patients with sickle cell anemia and 45 healthy individuals. For all participants, their pure tone hearing thresholds and videonystagmography (VNG) findings have been determined in 17 frequencies between 125–16.000 Hz. Results: All hearing thresholds between 125 and 16,000 Hz, pure tone averages of patients with sickle cell anemia have been found statistically significant to be higher than the corresponding values in the control group(p < 0.05). The normal hearing rate of patients with sickle cell anemia has been determined to be 71.1% conductive hearing loss (CHL) to be 4.4%, sensorineural hearing loss (SNHL) to be 22.2%, and mixed type hearing loss to be 2.2% in right ear; the normal hearing rate has been determined to be 71.1%, CHL to be 2.2%, SNHL to be 22.2%, and mixed type hearing loss to be 4.4% in left ear. Statistically significant difference has not been found between head shake, spontaneous nystagmus, optokinetic, tracking test batteries, static and dynamic positional tests used in VNG, saccade accuracy and saccade peak velocity, which are saccadic test findings of 2 groups. However, saccadic latency, which is a saccadic test finding, has been determined to be longer in patients with sickle cell anemia in comparison to the control group. Conclusion: While sickle cell anemia causes hearing deficits, it does not have any effect on the central or peripheral vestibular system.

scholarly journals Evaluation of the Relationship Between the Air–Bone Gap and Prolonged ABR Latencies in Mixed-Type Hearing Loss

2017 ◽  
Vol 13 (1) ◽  
pp. 88-92
Author(s):  
Omer Faruk Birkent ◽  
Turgut Karlidag ◽  
Figen Basar ◽  
Sinasi Yalcin ◽  
Irfan Kaygusuz ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Mixed Type ◽  
The Relationship

scholarly journals A Father and Son with a Nonsevere form of Crouzon's Syndrome

2000 ◽  
Vol 79 (5) ◽  
pp. 368-371
Author(s):  
Bülent Mamikoglu ◽  
Aysegül Mamikoglu
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Mixed Type ◽  
Autosomal Dominant ◽  
Cranial Sutures ◽  
Autosomal Dominant Disorder ◽  
Classic Form ◽  
Patients Experience ◽  
Father And Son ◽  
Crouzon's Syndrome

Crouzon's syndrome is a hereditary autosomal-dominant disorder. In its classic form, patients experience a premature closure of the cranial sutures, which leads to brachycephaly, proptosis, a small maxilla, and anomalies of the external and middle ear. In this report, we describe the case of a father and son who both had a nonsevere form of this disorder. The two men did not have brachycephaly or proptosis, but they did have ptosis and a mixed-type hearing loss.

Progressive Hearing Loss from Strial Dysplasia

1987 ◽  
Vol 96 (2) ◽  
pp. 229-231
Author(s):  
Sho Hashimoto ◽  
Harold F. Schuknecht
Keyword(s):  
Early Childhood ◽  
Hearing Loss ◽  
Male Patient ◽  
Mixed Type ◽  
Healthy Male ◽  
Progressive Hearing Loss ◽  
Temporal Bones ◽  
Genetically Determined

An otherwise healthy male patient had a bilateral, slowly progressive hearing loss first noticed in early childhood and possibly present at birth. Audiometric studies at the age of 32 showed a moderately severe, bilateral, mixed type hearing loss. He died of unrelated causes at the age of 34. Studies of the temporal bones showed bilateral hypoplasia and atrophy of the striae vascularis as the cause of hearing loss. The history and findings are consistent with a genetically determined hypoplasia and atrophy of this structure.

Hearing impairment in unilateral chronic suppurative otitis media

1986 ◽  
Vol 67 (5) ◽  
pp. 370-372
Author(s):  
A. Ya. Nugumanov
Keyword(s):  
Hearing Loss ◽  
Hearing Impairment ◽  
Otitis Media ◽  
Mixed Type ◽  
Chronic Suppurative Otitis Media ◽  
Auditory Function ◽  
Suppurative Otitis Media ◽  
Long Time

When studying the auditory function of patients suffering from unilateral chronic purulent otitis media for a long time, we noticed that along with hearing reduction in the diseased ear of the mixed type, there was often hearing reduction in the opposite ear of the neurosensory hearing loss type. These changes were even more pronounced when chronic purulent otitis media was combined with labyrinthitis. In patients aged 50 years and older, hearing loss in the opposite ear, exceeding age thresholds, was more frequent.

Otolaryngologic Features of Craniometaphyseal Dysplasia

1987 ◽  
Vol 96 (6) ◽  
pp. 548-553 ◽  
Author(s):  
G. Robert Kletzker ◽  
Peter G. Smith ◽  
Thomas E. Killeen
Keyword(s):  
Hearing Loss ◽  
Facial Nerve ◽  
Facial Paralysis ◽  
Mixed Type ◽  
Operative Management ◽  
Disease Group ◽  
Usual Symptom ◽  
Cranial Neuropathies ◽  
Nerve Dysfunction ◽  
Craniometaphyseal Dysplasia

Craniometaphyseal dysplasia, one of several rare familial systemic bone disorders of the osteopetrosis bone-disease group, is characterized by abnormal bony thickening of the skull and multiple cranial neuropathies. Hearing loss, commonly of a mixed type, is the usual symptom presented to the otolaryngologist—head and neck surgeon; however, recurring facial paralysis, blindness, and atypical fades may also be noted. A case of bilateral facial nerve dysfunction in a neonate, the offspring of a thoroughly studied kindred with known craniometaphyseal dysplasia, Is presented. The clinical and radiographic features of the disorder are discussed, including an outline of the operative management of facial paralysis.

A new syndrome of microtia with mixed type hearing loss, renal agenesis, and multiple skeletal anomalies

2006 ◽  
Vol 140A (7) ◽  
pp. 747-751 ◽  
Author(s):  
Yavuz Demir ◽  
Hale Samli ◽  
Aylin Yucel ◽  
M. Deniz Yilmaz ◽  
Nurten Turhan Haktanir ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Mixed Type ◽  
Renal Agenesis ◽  
Skeletal Anomalies

AUDIOLOGICAL FINDINGS IN HEARING LOSS SECONDARY TO MATERNAL RUBELLA

PEDIATRICS ◽  
1970 ◽  
Vol 45 (2) ◽  
pp. 225-229
Author(s):  
Thomas E. Borton ◽  
Earl W. Stark
Keyword(s):  
Hearing Loss ◽  
Hearing Impairment ◽  
Mixed Type ◽  
Congenital Abnormalities ◽  
First Trimester ◽  
Congenital Defects ◽  
Careful Attention ◽  
Hearing Impairments ◽  
First Trimester Of Pregnancy ◽  
Almost All

The purpose of this investigation was to describe the audiological findings in hearing loss secondary to maternal rubella. The mothers of all but three of the 80 subjects contracted rubella during the first trimester of pregnancy. Over 75.0% sustained the infection during the second or third month of gestation. The greatest incidence of additional congenital defects was found in those instances in which maternal rubella occurred during the first month of pregnancy. In almost all instances, all other congenital abnormalities had been identified prior to diagnosis of hearing impairment. Therefore, careful attention should be given to the possibility of undiagnosed hearing impairment being present in children whose mothers contracted rubella during the time of pregnancy. Audiograms were available for 55 of the subjects. The majority of these 55 subjects demonstrated hearing impairments classified as moderately severe to profound in degree. Ear differences were found in only 16 (29.0%) of the 55 children. No audiometric configuration emerged as representative of hearing impairment secondary to maternal rubella. Nevertheless, 40% of the audiograms were falling in pattern. The majority of the subjects demonstrated sensorineural impairments. However, about one-fourth exhibited mixed-type losses, according to the audiogram.

Otological findings in idiopathic hyperphosphatasia

1999 ◽  
Vol 113 (2) ◽  
pp. 158-160 ◽  
Author(s):  
Levent Sennaroǧlu ◽  
Bulent Sozeri ◽  
Arzu Sungur
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Mixed Type ◽  
Ossicular Chain ◽  
Otic Capsule ◽  
Progressive Hearing Loss ◽  
Middle Ear Mucosa ◽  
Superior Part ◽  
Bony Mass ◽  
Temporal Bones

AbstractA 17-year-old male patient was admitted because of progressive hearing loss since the age of six. His former blood and radiology investigation had revealed idiopathic hyperphosphatasia. On ENT examination bilateral thickened tympanic membranes with severe mixed-type hearing loss was diagnosed. Computerized tomography (CT) demonstrated expansion of the calvarial bones, including the temporal bones, except for the otic capsule. Middle-ear exploration revealed thickened middle-ear mucosa and a stone hard, immobile bony mass instead of the normal ossicular chain at the posterior superior part of the mesotympanum. No ossicular reconstruction could be attempted and the patient was rehabilitated with a hearing aid.

Progressive Mixed Type Hearing Loss in an Adult Male with Osteopetrosis

2009 ◽  
Vol 119 (S1) ◽  
pp. S39-S39
Author(s):  
John P. Gniady ◽  
Max L. Ronis
Keyword(s):  
Hearing Loss ◽  
Adult Male ◽  
Mixed Type
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