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Malleus head fixation: association with pseudohypoparathyroidism
M J LaRouere1, G Coleman, S A Telian
1Department of Otolaryngology-Head and Neck Surgery, University of Michigan Hospitals, Ann Arbor 48109.
The American Journal of Otology
|September 1, 1990
Summary
Pseudohypoparathyroidism (PHP) causes ectopic calcification, potentially affecting the malleus bone. This case report details the first instance of malleus head fixation in PHP, noting that metabolic correction does not reverse the condition.
Area of Science:
- Endocrinology
- Otolaryngology
- Genetics
Background:
- Pseudohypoparathyroidism (PHP) is characterized by resistance to parathyroid hormone (PTH), leading to hypocalcemia and hyperphosphatemia.
- PHP can manifest with ectopic calcifications in various tissues, including the head and neck.
- Known head and neck issues include tetany, laryngeal spasm, and calcification in ocular, dental, subcutaneous, and brain tissues.
Observation:
- This report details the first documented case of malleus head fixation due to ectopic calcification in a patient with Pseudohypoparathyroidism.
- The calcification specifically affected the malleus head, a small bone in the middle ear.
Findings:
- Ectopic calcification in Pseudohypoparathyroidism can manifest in the head and neck, including the middle ear structures.
- Normalization of serum calcium and phosphate levels did not prevent or reverse the malleus head fixation.
Implications:
- Malleus head fixation represents a novel head and neck manifestation of Pseudohypoparathyroidism.
- Surgical interventions such as ossiculoplasty or hearing aids are the current recommended treatments for hearing impairment caused by this condition.