90 Copyright © 2011 The Korean Audiological Society CASE REPORT
Korean J Audiol 2011;15:90-93 pISSN 2092-9862 / eISSN 2093-3797
Introduction
Histiocytosis is a group of proliferative disorders character- ized by the infiltration and accumulation of histiocytes and other immune effector cells within various tissues.1) The term histiocyte includes monocytes and macrophages, dermal/inter- stitial dendritic cells, and Langerhans cells. Langerhans cell histiocytosis (LCH) is characterized by the aberrant prolifera- tion of Langerhans cells normally found in the dermis.2) It fre- quently affects skull base areas, mainly temporal bones and frontal bones. In cases involving temporal bones, LCH is fre- quently located in the mastoid process and can infiltrate into the middle and external ears. It displays diverse symptoms de- pending on the site of involvement.3) But, specific symptoms and treatments have not been reported.
We experienced a case of external auditory canal stenosis in LCH with multiple organ involvement in a 28-year-old male, and report it with the review of the relevant literature.
Case Report
A 28-year-old male who had suffered from hearing im- pairment in the right side for 3 days was first observed in our outpatient clinic. The patient took medications for bilateral
chronic otitis media about 10 years ago, and, for the past 2 years, had been undergoing chemotherapy and radiotherapy due to multifocal LCH involving the scalp, the axilla, and the lung. There was no symptom except right side hearing impairment. Both external auditory canals were nearly ob- structed and eardrums were not observed. Bilateral otorrhea and granulomas in the external auditory canal were observed, but swelling of the posterior auricular area was not observed.
Initial hearing test showed normal hearing thresholds in both ears (Fig. 1A). A computed tomography scan revealed obstructed bilateral external auditory canals with soft tissue density lesions, but the mastoid and the middle ear were clear (Fig. 2). The diagnosis of LCH was confirmed by skin biopsy in the outpatient clinic. The pathologic findings showed proliferative Langerhans cells in the dermis and posi- tive staining with S-100 and CD1a in immunohistochemistry (Fig. 3A, B and C). T-tubes were inserted in both external auditory canals (Fig. 3D) and radiotherapy was commenced.
However, both external auditory canals were totally ob- structed due to progression of the disease 6 months after start- ing the treatment. Two years after the first visit, bilateral con- ductive hearing loss was discovered (air-bone gaps, 45 dBHL)(Fig. 1B). Therefore we planned surgical treatment to maintain patency of the external auditory canal. First, a right
A Case of External Auditory Canal Stenosis in Langerhans Cell Histiocytosis
Inkyung Sohn, Han-Bin Lee, Sung-Yeul Kim and Yun-Hoon Choung
Department of Otolaryngology, Ajou University School of Medicine, Suwon, Korea
Received May 26, 2011 Revised June 29, 2011 Accepted June 30, 2011 Address for correspondence Yun-Hoon Choung, MD, DDS, PhD Department of Otolaryngology, Ajou University School of Medicine, San 5 Woncheon-dong, Yeongtong-gu, Suwon 443-721, Korea
Tel +82-31-219-5263 Fax +82-31-219-5264 E-mail [email protected]
Langerhans cell histiocytosis (LCH) refers a group of disorders of the reticuloendothelial sys- tem characterized by a proliferation of histiocytes, which includes eosinophilic granuloma, Let- tere-Siwe diseases, and Hand-Schuler Christian disease. The clinical presentation of LCH de- pends on the site of involvement. It can range from multifocal lesions to a solitary lesion.
Tissues characteristically involved in LCH are bone, skin, lung, liver, spleen, bone marrow, lymph nodes and the hypothalamic-pituitary region, although the involvement of other organs such as the bowel can occur. We experienced a case of external auditory canal stenosis in LCH of multiple organ involvement in a 28-year-old male, and report it with a review of the rel-
evant literature. Korean J Audiol 2011;15:90-93
KEY WORDS: Langerhans cell histiocytosis · Temporal bone · External auditory canal · Stenosis.
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side tumor excision was done; the left side was performed 4 months later. A circumferential incision was made on the site about 5 mm medial from the external auditory canal open- ing, and granulation tissue and skin adhering to the tumor were completely removed. A part of the bony external audi- tory canal was exposed, but drills were not used. The ex- posed bone was covered with gelfoam and packed with ear Merocels. Merocels were replaced with Nelaton catheters 1 week after the surgery to prevent growth of granulation tis- sue (Fig. 3E). Three months after the surgery, obstruction re- occurred due to recurrence of the disease, and Nelaton cath- eters were frequently changed because of discharge and granulation tissue. Finally, 3 years and 6 months after the surgery, both external auditory canals were totally obstructed again (Fig. 3F, G). The last hearing test showed conductive hearing loss (air-bone gaps, 35 dBHL), but the patient is not undergoing any treatment for obstruction of the external audi- tory canal and hearing impairment at present. Treatment with Talidomide (50 mg qd) was recently tried in the Depart- ment of Oncology because of a new exacerbation of LHC with skin and oral involvement.
Discussion
LCH is an abnormal proliferative disease of histiocytes in- cluding eosinophillic granuloma, Hand-Schuller-Christian dis- ease, and Letterer-Siwe disease. In 1953, Lichtenstein described them together as ‘Histiocytosis X-related manifestations of a single nosologic entity’.2) The incidence of LCH is around 5.4 per million and it is predominantly found in males. Its peak in- cidence period is noted in children 1-4-years-of-age, but it can affect patients of any age.4) Its cause has not been defined yet. It can range from multifocal lesions to a solitary lesion.
In 60% of cases, it affects the skull base as a solitary lesion.
Cases involving temporal bone account for 19-25% of cas- es, and about one-third display bilateral involvement.4) In cases involving temporal bone, graulomas cause obstruction of the bony external auditory canal, intractable otorrhea and pain. It is often misdiagnosed because the clinical presenta- tion may mimic more common conditions like mastoiditis, chronic otitis media, or recurrent otitis externa.5) Hearing loss can range from mild conductive hearing loss to severe senso- rineural hearing loss according to the extent of the disease.
Fig. 2. Preoperative computed to- mography of temporal bone show- ing that both external auditory ca- nals are obstructed by lesions with soft tissue density (arrows). A: Axial
view. B: Coronal view. A B
Fig. 1. Pure tone audiograms show- ing normal hearing level in both ears at the first visit to the clinic (A) and both conductive hearing loss at the
2-year follow-up (B). A B
-10 0 10 20 30 40 50 60 70 80 90 100 110
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125 250 500 1 K 2 K 4 K 8 K 110 125 250 500 1 K 2 K 4 K 8 K
92 Korean J Audiol 2011;15:90-93 Auditory in Langerhans Cell Histiocytosis
Although conductive hearing loss is the most common prob- lem, permanent sensorineural hearing loss may result from damage to the inner ear and bony labyrinth.2) LCH has an un- predictable natural history varying from a rapid progressing and fatal disease to spontaneous resolution. Poor prognostic factors are age at presentation (younger than 2-years-of-age), multisystem involvement and vital organ dysfunction.4) Diag- nosis of LCH is confirmed by the characteristic morphology and the presence of CD1a or CD207 (langerin) positive his- tiocytic cells. Nowadays, electron microscopic confirmation of the presence of Birbeck granules-immature Langerhans cell with characteristic pentalaminar organelles is rarely used.2)
In this case, the patient had a history of multifocal histiocy- tosis and revealed bilateral otorrhea and stenosis of the exter- nal auditory canal. A computed tomography scan showed a soft tissue density lesion in both external auditory canals with- out bony destruction. Therefore, LCH was suspected, and was confirmed by skin biopsy. Comparatively young age of pre- sentation and multifocal lesions predict poor prognosis.
There are several treatment modalities for temporal bone LCH, including surgery, radiotherapy, chemotherapy and in- jection of steroids.4) These treatments can be used alone or in combination depending upon the extent and severity of the disease. The preferred treatment is surgical removal when pos-
sible.5,6) Chemotherapy is helpful in a multifocal disease or when lesions are inaccessible to local treatment.4) Solitary ra- diotherapy is no longer recommended, but it is used as an ad- ditional treatment for residual lesions after surgical exci- sion.4,7-9)
The major symptom of this case was hearing impairment due to stenosis of the external auditory canal. Surgical exci- sion and insertion of T-tubes and Nelaton catheters were tried, but the effect was transient. At the present time, both external auditory canals are completely obstructed, and there is no in- terval change in conductive hearing loss. Although the pre- ferred treatment for a localized lesion is surgical excision, it is a limited treatment unless preventive measures of obstruction are not developed. New techniques or methods are needed to prevent restenosis of the external auditory canal due to postop- erative radiotherapy or tumor expansion.
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Fig. 3. Langerhans cells are concentrated in dermis (star) under external auditory canals (EAC, arrow) in hematoxilin and eosin stain- ing (A, ×200). Langerhans cells (brown) are stained for s100 protein (B, ×400) and CD1a (brown) (C, ×400). T-tube (D) applied to in- hibit the expansion of tumors in the EAC was ineffective. After resection of tumors, a Nelaton catheter (width: 10 Fr. length: 1.5 cm)(E) was inserted to maintain the patency of the EAC. However, both EACs were totally obstructed by tumors again (F: Right, G: Left).
EAC: external auditory canal.
A B C
E D
F G
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