Robert A. Jahrsdoerfer

Aspergillosis of the nose and paranasal sinuses

Aspergillosis of the nose and paranasal sinuses should not be considered an innocuous disease. In a review of 103 cases, other than in the Sudan, the mortality rate was 16 per cent. Intracranial extension of the fungus from the nose and sinuses carries a grave prognosis. Although aspergillosis occurs frequently in patients debilitated from other diseases, this is not always the case, and most deaths in the review series occurred in otherwise healthy individuals. The authors present eight cases of their own.

CT evaluation of congenital aural atresia: what the radiologist and surgeon need to know.

PURPOSE The preoperative evaluation of aural atresia and stenosis is strongly dependent upon high resolution CT. We have devised a 10 point surgical rating scale based on high resolution CT of the temporal bone that will provide radiologists with a stepwise method of evaluating these scans and will allow them to communicate these findings to otologic surgeons in a consistent fashion. METHOD We prospectively evaluated 1,500 patients with aural atresia or stenosis for eight critical areas of temporal bone anatomy, each area receiving 1 rating scale point, with the exception of the presence of a stapes, which received 2 points. The appearance of the external ear received the final point on the rating scale since the external ear is formed earlier than the middle ear and helps to predict its deformities. The presurgical score was utilized in selecting surgical candidates and was correlated with the intraoperative findings as well as the postsurgical results by comparing pre- and postoperative speech reception threshold. Patients with a presurgical rating of < or = 5 were not considered surgical candidates. Otherwise, the percentage of successful surgeries corresponded roughly to the rating scale. RESULTS AND CONCLUSION Thus, a presurgical rating of 8 points translates into an 80% chance of restoring hearing to normal or near-normal levels.

Labyrinthine trauma during ear surgery.

Surgical trauma inflicted upon the membranous labyrinth is an uncommon, but often catastrophic, complication of chronic ear surgery. Areas most vulnerable to injury are the oval window and the lateral semicircular canal. Methods of injury include subluxation of the footplate, fracture of the lateral canal by drill or chisel, and accidental opening of a fistula.

Congenital Malformations of the Ear Analysis of 94 Operations

The 94 operations analyzed were performed on 80 ears in 73 patients with congenital malformation of the ear. Operative findings included 13 patients with a congenital absence of the oval window, 5 patients with vascular anomaly of the middle ear, and 6 patients with congenital cholesteatoma. Six of 13 patients with congenital absence of the oval window had a labyrinthotomy performed to create a new opening into the vestibule. The method of surgical repair in atresia was a canalplasty with fascia graft overlay for the substitute tympanic membrane. A Silastic® button was placed over the substitute tympanic membrane to lessen blunting and to anchor the center-hole skin graft in place. A modified face lift procedure was done if necessary to align the external ear with the new ear canal. At present no ear canals are stenosed and no ears are actively discharging. One patient sustained a temporary facial paralysis. The hearing results are discussed.

Conservative Surgical Treatment of Chondrosarcoma of the Larynx

Chondrosarcoma of the larynx is a rare malignant tumor sometimes encountered by otolaryngologists. Since they are usually of low grade malignancy, total laryngectomy is indicated only in cases of high grade malignancy or where total excision by conservative (partial) laryngectomy is not feasible. This paper reviews chondrosarcoma of the larynx, discusses a patient who developed this tumor, and describes a method of conservative surgical excision and laryngotracheal reconstruction when the tumor involves the cricoid, the most common site of occurrence in the larynx.

Endolymphatic Duct Obstruction from a Jugular Bulb Diverticulum

Herniation of the jugular bulb into the middle ear is uncommon. Extension of a jugular bulb diverticulum into the petrous pyramid is rare. In ten previously reported cases in the literature, only the radiographic appearance of the lesion was stressed. Of interest to otolaryngologists is the fact that in seven of the cases there was an ipsilateral sensorineural hearing loss. Other symptoms included tinnitus and vertigo. One patient had been diagnosed as having Menières disease. We document an additional case (a patient who had “classical Menières disease”) and report the first known surgical exploration of this venous anomaly. The diverticulum extended high into the petrous bone and was found to obliterate the distal segment of the endolymphatic duct. To our knowledge, this is the first time that a cause and effect relationship has been recognized between endolymphatic hydrops and a jugular bulb diverticulum. We propose that the diverticulum, by obstructing the endolymphatic duct, was directly responsible for the Menières-like symptoms.

Subcochlear Approach for Cholesterol Granulomas of the Inferior Petrous Apex

Cholesterol granulomas of the petrous apex are drained through two major extra-labyrinthine routes: One, along the posterosuperior chain of air cells, and two, along the anteroinferior chain. Procedures that use the posterosuperior chain approach the apex from the sinodural angle, the base of the zygomatic arch, the attic, or through the arch of the superior semicircular canal. Operations that use the anteroinferior chain reach the apex along the internal carotid canal (Ramadlers operation) or by a posterior infralabyrinthine approach between the descending facial nerve and jugular bulb. Inferior petrous apex cholesterol granulomas may be unreachable by any of these routes, and hence the subcochlear route is proposed as an alternative. The subcochlear approach starts in a triangle bounded superiorly by the cochlea, anteriorly by the internal carotid canal and posteriorly by the deep jugular vein. This operation requires lowering the inferior bony canal wall to the level of the “crutch.” It provides access to an inferiorly situated cholesterol granuloma, yet preserves hearing. It allows enough room for the placement of a tube drain from the petrous apex to the mastoid. It is particularly useful when a high jugular bulb precludes the use of the posterior infralabyrinthine route.

The facial nerve in congenital middle ear malformations

The two most common anomalies of the facial nerve encountered in patients with a congenital malformation of the middle ear are displacement of the nerve and lack of a bony cover, two conditions that place the nerve at risk of being injured by the unwary surgeon. Malformations of the stapes are often found in association with facial nerve anomalies and may range from underdevelopment to complete absence. A congenital absence of the oval window is not uncommon.

Otitis media and the immotile cilia syndrome

The immotile cilia syndrome appears to be a congenital defect in the ultrastructure of cilia that renders them incapable of movement. Respiratory tract cilia and sperm are predominantly affected. Bronchiectasis, sinusitis and male sterility are the main clinical findings. Situs inversus may be found. To these findings can be added otitis media.

Treacher Collins syndrome: an otologic challenge

Patients with Treacher Collins syndrome have severe middle ear malformations that render operation difficult. We have evaluated 43 patients with Treacher Collins syndrome, on whom only 11 were operated. Computed tomography, the single most important study done preoperatively, routinely showed an underdeveloped temporal bone with islands of bone marrow and absent mastoid pneumatization. The middle ear space was often underdeveloped. Ossicular dysjunction was often noted in which the fused malleus/incus remnant was found 3 to 4 mm distant to the stapes. A common finding was severe dysplasia of the stapes-facial nerve complex that often made the middle ear malformation uncorrectable. Hearing results were much less predictable than in patients with isolated atresia/stenosis of the ear.