Spectrum of Middle and Inner Ear Abnormalities in Infants With Congenital Heart Defects

Objective: To evaluate temporal bone histopathology in infants with congenital heart defects (CHD). Study Design and Setting: A retrospective review of our temporal bone collection was conducted to identify temporal bones acquired from infants with CHD. Subjects were divided into nonsyndromic and syndromic CHD groups. The presence of temporal bone abnormalities and the incidence of abnormalities that may result in hearing impairment were determined. Results: Thirty-eight temporal bones obtained from 16 infants with nonsyndromic CHD and 4 with syndromic CHD were evaluated. Nonsyndromic CHD cases had abnormalities such as a mesenchymal remnant, malformed stapes, persistent stapedial artery, shallow round window, dehiscent facial nerve canal, short cochlea, strial basophilic deposits, deformity of the spiral ligament, bulging Reissner's membrane, hypoplastic lateral semicircular canal, and cupular deposits. Syndromic CHD cases had abnormalities including narrow round window niche, facial canal dehiscence, strial basophilic deposits and cysts, and outer hair cell loss. Middle and inner ear abnormalities that may impair hearing were observed in 6 subjects with nonsyndromic CHD and in 1 subject with syndromic CHD. Conclusions: A wide variety of temporal bone defects were documented in infants with CHD. Congenital middle and inner ear abnormalities should be anticipated in the hearing assessment and otologic surgery of infants with CHD.

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Otologic Histopathology of Fabry's Disease

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348948909800509 ◽

1989 ◽

Vol 98 (5) ◽

pp. 359-363 ◽

Cited By ~ 43

Author(s):

Patricia A. Schachern ◽

Michael M. Paparella ◽

Donald A. Shea ◽

Tae H. Yoon

Keyword(s):

Temporal Bone ◽

Ganglion Cells ◽

Spiral Ganglion ◽

Cell Loss ◽

The Body ◽

Spiral Ligament ◽

Fabry’S Disease ◽

Fabry's Disease ◽

Temporal Bones ◽

Spiral Ganglia

Fabry's disease is a rare progressive X-linked recessive disorder of glycosphingolipid metabolism. The accumulation of glycosphingolipids occurs in virtually all areas of the body, including the endothelial, perithelial, and smooth-muscle cells of blood vessels, the ganglion cells of the autonomic nervous system, and the glomeruli and tubules of the kidney. Although otologic symptoms have been described in these patients, to our knowledge there have been no temporal bone histopathologic reports. We describe the clinical histories, audiometric results, and temporal bone findings of two patients with this rare disorder. Both patients demonstrated a bilateral sloping sensorineural hearing loss audiometrically. Middle ear findings of seropurulent effusions and hyperplastic mucosa were seen in all four temporal bones. Strial and spiral ligament atrophy in all turns, and hair cell loss mainly in the basal turns, were also common findings. The number of spiral ganglion cells was reduced in all temporal bones; however, evidence of glycosphingolipid accumulation was not observed in the spiral ganglia.

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Temporal Bone Pathology in Scuba Diving Deaths

Otolaryngology ◽

10.1177/019459989310900321 ◽

1993 ◽

Vol 109 (3) ◽

pp. 514-521 ◽

Cited By ~ 15

Author(s):

Patrick J. Antonelli ◽

G. Joseph Parell ◽

Gary D. Becker ◽

Michael M. Paparella

Keyword(s):

Inner Ear ◽

Temporal Bone ◽

Round Window ◽

Scuba Diving ◽

Round Window Membrane ◽

Bone Pathology ◽

Rapid Ascent ◽

Temporal Bones ◽

Inner Ear Damage ◽

Air Cells

Scuba diving has long been associated with otologic injuries; however, little is known about temporal bone pathology in diving-related deaths. We examined 18 temporal bones from 11 divers who died, primarily from complications of rapid ascent. Bleeding into the middle ear and mastoid air cells was nearly universal. Inner ear damage included hemorrhage around Reissner's membrane and the round window membrane and rupture of the utricle and saccule. Most of the observed inner ear damage was not surgically treatable. (OTOLARYNGOL HEAD NECK SURG 1993;109:514-21.)

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Temporal Bone Findings in Cases of Salt Water Drowning

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348948309200207 ◽

1983 ◽

Vol 92 (2) ◽

pp. 134-136 ◽

Cited By ~ 12

Author(s):

George Kelemen

Keyword(s):

Inner Ear ◽

Temporal Bone ◽

Middle Ear ◽

Salt Water ◽

Round Window ◽

Scuba Diving ◽

Serial Sections ◽

Histopathological Findings ◽

Histopathological Features ◽

Temporal Bones

The otologic literature contains no description of the histopathological features of temporal bones of persons who perished in drowning accidents. Three temporal bones from two fatal cases of drowning were studied in serial sections. Two temporal bones were from a man aged 31 years who collapsed after scuba diving and died three days later. One temporal bone was from a 32-year-old man, serving in the navy, who drove his car, probably accidentally, into the sea and drowned in it. Histopathological findings appeared to be similar in the two cases. Diffuse hemorrhage was evident throughout the middle ear and inner ear. The tympanic and round window membranes appeared to be intact although hemorrhage had occurred within the tissue layers of the membranes. This finding contrasts with observations of ruptured membranes by other investigators.

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Spectrum of Middle and Inner Ear Abnormalities in Infants With Congenital Heart Defects

Yearbook of Otolaryngology-Head and Neck Surgery ◽

10.1016/s1041-892x(08)70031-3 ◽

2006 ◽

Vol 2006 ◽

pp. 32-33

Author(s):

M.M. Paparella

Keyword(s):

Inner Ear ◽

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Defects

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Temporal Bone Histopathology in Charge Association

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348940211100503 ◽

2002 ◽

Vol 111 (5) ◽

pp. 397-401 ◽

Cited By ~ 13

Author(s):

Shin-Ichi Haginomori ◽

Makoto Miura ◽

Isamu Sando ◽

Margaretha L. Casselbrant

Keyword(s):

Inner Ear ◽

Temporal Bone ◽

Middle Ear ◽

Congenital Heart ◽

The Other ◽

Otologic Surgery ◽

Charge Association ◽

Ear Anomalies ◽

Temporal Bones ◽

Central Nervous System Anomalies

Three temporal bones obtained at autopsy from 2 patients with CHARGE association (Coloboma, congenital Heart disease, Atresia of choanae, mental Retardation and/or central nervous system anomalies, Genital hypoplasia, and Ear anomalies) were examined histopathologically. Both temporal bones from 1 patient showed multiple anomalies in the middle ear, inner ear, and facial nerve. However, the temporal bone obtained from the other patient showed almost normal structures in the inner ear, middle ear, and eustachian tube. These results are different from those of 2 previous reports of temporal bone histopathology regarding CHARGE association. This difference suggests that CHARGE association may arise not from one etiopathogenetic factor, but from complex factors. Special attention to dehiscent facial canal and perilymphatic gusher during otologic surgery in patients with CHARGE association is discussed.

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